autoimmune oophoritis

Autoimmune oophoritis is a rare cause of primary ovarian insufficiency (POI). It happens when the body's immune system mistakenly attacks the ovaries causing inflammation, atrophy and fibrosis. These changes stop the ovaries from working normally. The main symptoms of autoimmune oophorotis are irregular or absent menstrual period (amenorrhea) and symptoms related to ovarian cysts such as abdominal cramping, bloating, nausea and vomiting. Autoimmune oophoritis may occur as part of autoimmune polyglandular syndrome type I and type II but has also been associated with lupus, pernicious anemia, myasthenia gravis and other autoimmune conditions. The underlying cause of autoimmune oophoritis is unknown. Diagnosis involves a special blood test which looks for anti-steroid or anti-ovarian antibodies, a pelvic ultrasound to look for enlarged cystic ovaries and tests to rule out other possible causes of POI. Management of autoimmune oophoritis involves emotional support, possible estrogen replacement therapy and management of other autoimmune conditions.


atrophic thyroiditis

biolink:Disease biolink:DiseaseOrPhenotypicFeature biolink:BiologicalEntity biolink:NamedThing biolink:Entity biolink:ThingWithTaxon

Atrophic thyroiditis is an organ-specific autoimmune disease characterized by thyroid autoantibodies, functional hypothyroidism, and absence of goiter.


primary hypophysitis

Immune-mediated inflammation of the pituitary gland often associated with other autoimmune diseases (e.g., hashimoto disease; graves disease; and addison disease).


autoimmune primary ovarian failure

An autoimmune form of primary ovarian failure.


postpartum thyroiditis

A transient autoimmune Inflammatory disorder of thyroid gland that occurs postpartum due to any partum problem. It is characterized by the presence of high titers of autoantibodies against thyroid peroxidase and thyroglobulin. Clinical signs include the triphasic thyroid hormone pattern: beginning with thyrotoxicosis, followed with hypothyroidism, then return to euthyroid state by 1 year postpartum


IgG4-related thyroid disease

Riedel thyroiditis is a fibroinflammatory disorder of the thyroid gland, occuring more frequently in females, characterized a large, hard thyroid mass, and presenting with pressure symptoms (breathing difficulB,ties and dysphagia) or voice hoarseness and aphonia (impingement of recurrent laryngeal nerve). It can often be associated with extracervical fibroinflammatory disorders such as retroperitoneal fibrosis, primary scleroisng cholangitis and autoimmune diseases such as Hashimoto struma, Addison disease, and Biermer disease.


autoimmune polyendocrinopathy

A group of diverse conditions that are characterized by spontaneous, multi-organ autoimmunity, which target both endocrine (adrenal, gonad, pancreatic islet cells, parathyroid, pituitary, thyroid) and non-endocrine (gastrointestinal, integumentary, lymphatic) tissues.


autoimmune polyendocrinopathy type 2

Autoimmune polyglandular syndrome of likely polygenic etiology characterized by the presence of primary adrenal insufficiency in association with autoimmune thyroiditis and/or type 1 diabetes mellitus; this condition is not associated with mucocutaneous candidiasis.


autoimmune polyendocrine syndrome type 1

Autoimmune polyendocrinopathy type 1, or APECED syndrome, is a genetic disease that manifests in childhood or early adolescence with a combination of chronic mucocutaneous candidiasis, hypoparathyroidism and autoimmune adrenal failure.


Abediterol

A quinolone that is 8-hydroxyquinolin-2(1H)-one which carries a 2-{[6-(2,2-difluoro-2-phenylethoxy)hexyl]amino}-1-hydroxyethyl group at position 5. It is a long acting beta2-adrenoceptor agonist currently in development for the treatment of chronic obstructive pulmonary disease (COPD) and asthma.


AV Block First Degree by ECG Finding

biolink:PhenotypicFeature biolink:DiseaseOrPhenotypicFeature biolink:BiologicalEntity biolink:NamedThing biolink:Entity biolink:ThingWithTaxon

An electrocardiographic finding of prolonged PR interval for a specific population. For adults one common threshold is a PR interval greater than 0.20 seconds. Note that other thresholds may be applicable. (CDISC)


parathyroid hormone/parathyroid hormone-related peptide receptor

A parathyroid hormone receptor that is a translation product of the human PTH1R gene or a 1:1 ortholog thereof.


parathyroid hormone receptor

A secretin-like G-protein coupled receptor that is a translation product of the human PTH1R or PTH2R genes, 1:1 orthologs thereof, or a semi-ortholog thereof.


parathyroid hormone 2 receptor

A parathyroid hormone receptor that is a translation product of the human PTH2R gene or a 1:1 ortholog thereof.


parathyroid hormone 2 receptor, signal peptide removed form (human)

A parathyroid hormone 2 receptor (human) that has had the signal peptide removed. UniProtKB:P49190, 25-550.


parathyroid hormone/parathyroid hormone-related peptide receptor isoform 1 (rat)

A parathyroid hormone/parathyroid hormone-related peptide receptor isoform 1 that is encoded in the genome of rat.


parathyroid hormone/parathyroid hormone-related peptide receptor, signal peptide removed form (human)

A parathyroid hormone/parathyroid hormone-related peptide receptor (human) that has had the signal peptide removed. UniProtKB:Q03431, 27-593.


Unresectable NUT Carcinoma

NUT carcinoma that is not amenable to surgical resection.


A53 Diffuse Large B-Cell Lymphoma

A genetic subtype of diffuse large B-cell lymphoma characterized by TP53 gene mutations and deletions that is often accompanied by aneuploidy.


spindle cell nevus

A nevus characterized by the presence of spindle-shaped melanocytes.


Childhood Recurrent Malignant Solid Neoplasm

The reemergence of a childhood malignant solid neoplasm after a period of remission.


amelogenesis imperfecta type 2A1

Any amelogenesis imperfecta in which the cause of the disease is a mutation in the KLK4 gene.


Pelvic Floor Muscle Weakness

A reduction in the strength of the muscles of the pelvic floor.


Grade 1 Skin Papilloma, CTCAE

Asymptomatic; intervention not indicated


specific developmental disorder

A developmental disorder of mental health that categorizes specific learning disabilities and developmental disorders affecting coordination.


conduct disorder

A disorder diagnosed in childhood or adolescence age group characterized by aggressive behavior, deceitfulness, destruction of property or violation of rules that is persistent and repetitive, and within a one year period.


attention deficit-hyperactivity disorder 8


specific language impairment

A language disorder characterized by difficulty in language acquisition despite otherwise normal development and in the absence of any obvious explanatory factors.


fetal alcohol syndrome

Fetal alcohol syndrome (FAS) is a rare malformation syndrome caused by excessive maternal consumption of alcohol during pregnancy. It is characterized by prenatal and/or postnatal growth deficiency (weight and/or height <10th percentile), a unique cluster of minor facial anomalies (short palpebral fissures, flat and smooth philtrum, and thin upper lip) and severe central nervous system (CNS) abnormalities including microcephaly, and cognitive and behavioral impairment (intellectual disability, deficit in general cognition, learning and language, executive function, visual-spatial processing, memory, and attention).


mixed receptive-expressive language disorder

A disorder characterized by an impairment in the development of an individual's expressive and receptive language capabilities which is in contrast to his/her nonverbal intellect. The impairment may be acquired (i.e., due to a brain lesion or head trauma) or developmental (i.e., no known neurological insult).


transient tic disorder

A neurological disorder presenting in childhood that is characterized by motor and/or phonic tics that occur daily or nearly daily for one to twelve months and are not attributed to an identifiable cause.


articulation disorder

A disorder characterized by the failure to use developmentally expected speech sounds that are appropriate for the individual's age (i.e., the individual makes errors in sound production or use or omits sounds such as final consonants).


partial fetal alcohol syndrome

A fetal alcohol spectrum disorder that results in most, but not all, of the growth deficiency and/or craniofacial features of fetal alcohol syndrome including central nervous system dysfunction due to prenatal alcohol exposure.


dysgraphia

Loss or impairment of the ability to write (letters, syllables, words, or phrases) due to an injury to a specific cerebral area or occasionally due to emotional factors. This condition rarely occurs in isolation, and often accompanies aphasia. (From Adams et al., Principles of Neurology, 6th ed, p485; apa, Thesaurus of Psychological Index Terms, 1994)


specific language impairment 5

A communication disorder that involves the processing of linguistic information.


dyslexia

A learning disorder characterized by an impairment in processing written words. Reading difficulties can include distortions, omissions or substitutions of characters. Oral and silent reading difficulties can include faulty and slow comprehension.


A disorder characterized by a marked pattern of inattention and/or hyperactivity-impulsivity that is inconsistent with developmental level and clearly interferes with functioning in at least two settings (e.g. at home and at school). When present, the symptoms of hyperactivity are most often present before the age of 7 years. There are three recognized presentations or subtypes from most to least common: combined type, inattentive/distractible type, hyperactive/impulsive type.


fetal nicotine spectrum disorder

A specific developmental disorder that is characterized by physical, behavioral and learning birth defects resulting from maternal ingestion of nicotine during pregnancy.


writing disorder

A learning disability that involves impaired written language ability such as impairments in handwriting, spelling, organization of ideas, and composition.


expressive language disorder

A disorder characterized by an impairment in the development of an individual's expressive language which is in contrast to his/her nonverbal intellect and receptive language development. The impairment may be acquired (i.e., due to a brain lesion or head trauma) or developmental (i.e., no known neurological insult).


chronic tic disorder

A neurological disorder presenting in childhood that is characterized by either motor or phonic tics, but not both, that occur daily or nearly daily for at least a year and are not attributed to an identifiable cause.


alcohol-related birth defect

A physical or cognitive mental abnormality caused by maternal alcohol consumption and its toxic effect on the developing embryo during pregnancy.


fetal alcohol spectrum disorder

A group of disorders caused by a prenatal exposure to maternal consumption of alcohol leading to a range of behavioral, cognitive and neurological deficits in the offspring. It is characterized by physical growth problems, distinct facies, and varying psycho-neurological issues.


hereditary attention deficit-hyperactivity disorder

An instance of attention deficit-hyperactivity disorder that is caused by an inherited genomic modification in an individual.


neurobehavioral disorder with prenatal alcohol exposure

A fetal alcohol spectrum disorder that is characterized by one or more deficits in neurocognition and in self-regulation plus two or more deficits in adaptive functioning, with at least 1 in communication or social communication and interaction.


high affinity immunoglobulin gamma Fc receptor I isoform h2 (human)

A high affinity immunoglobulin gamma Fc receptor I (human) that is a translation product of some mRNA whose exon structure and start site selection renders it capable of giving rise to a protein with the amino acid sequence represented by UniProtKB:P12314-2.


Testicular Mixed Embryonal Carcinoma and Yolk Sac Tumor with Seminoma

A malignant germ cell tumor arising from the testis.  It is characterized by a mixture of embryonal carcinoma, yolk sac, and seminomatous morphologic elements.  Patients may present with painless or painful testicular swelling.


Lugano Classification Advanced Stage Adult Non-Hodgkin Lymphoma AJCC v8

Advanced stage adult non-Hodgkin lymphoma based on the Lugano classification criteria. It includes stages III and IV. (from AJCC 8th Ed.)


Advanced Non-Hodgkin Lymphoma

Non-Hodgkin lymphoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


Stage I Diffuse Large B-Cell Lymphoma

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Helicobacter Pylori-Associated Gastric Mucosa-Associated Lymphoid Tissue Lymphoma

A low grade, indolent B-cell lymphoma that is associated with Helicobacter pylori infection.


Intestinal T-cell lymphoma recurrent

The reemergence of enteropathy-associated T-cell lymphoma after a period of remission.


Adult T-Cell Leukemia/Lymphoma by Ann Arbor Stage

An anatomic stage for adult T-cell leukemia/lymphoma based on the Ann Arbor classification criteria.


Mycosis Fungoides and Sezary Syndrome by AJCC v7 Stage

A term that refers to the staging of mycosis fungoides and Sezary syndrome, following the rules of the TNM AJCC v7 classification system.


Primary Cutaneous B-Cell Non-Hodgkin Lymphoma

A B-cell non-Hodgkin lymphoma arising from the skin.  Representative examples include cutaneous marginal zone B-cell lymphoma and cutaneous follicle center lymphoma.


Refractory EBV-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified

EBV-positive diffuse large B-cell lymphoma, not otherwise specified that is resistant to treatment.


Central Nervous System Anaplastic Large Cell Lymphoma, ALK-Negative

An anaplastic large cell lymphoma, ALK-negative, arising from the central nervous system.


Stage IV Childhood Lymphoblastic Lymphoma

Stage IV: Tumors involve bone marrow and/or central nervous system (CNS) regardless of other sites of involvement (from PDQ).


Stage IVA1 Mycosis Fungoides and Sezary Syndrome AJCC v8

Stage IVA1 includes: T1-4, N0-2, M0, B2. T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface. T2: Patches, papules, or plaques covering 10% or more of the skin surface. T3: One or more tumors (equal or greater than 1 cm in diameter). T4: Confluence of erythema covering 80% or more of body surface area. N0: No clinically abnormal peripheral lymph nodes; biopsy not required. N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or National Cancer Institute (NCI) LN0-2. N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3. M0: No visceral organ involvement. B2: High blood tumor burden: 1,000 per microliter or more Sezary cells with positive clone. (AJCC 8th ed.)


Adult Burkitt Lymphoma

A Burkitt lymphoma occurring in adults.


Advanced Follicular Lymphoma

Follicular lymphoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


Recurrent Transformed Mycosis Fungoides

The reemergence of transformed mycosis fungoides after a period of remission.


Grade 1 Cutaneous Follicular Lymphoma

A cutaneous lymphoma which contains up to 5 centroblasts per 40X high-power microscopic field.


Recurrent Diffuse Large B-Cell Lymphoma Associated with Chronic Inflammation

The reemergence of diffuse large B-cell lymphoma associated with chronic inflammation after a period of remission.


Primary mediastinal large B-cell lymphoma stage IV

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Centroblastic lymphoma stage II

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE). The number of regions involved may be indicated by a subscript (e.g., II3).


Recurrent Breast Implant-Associated Anaplastic Large Cell Lymphoma

The reemergence of breast implant-associated anaplastic large cell lymphoma after a period of remission.


Primary Cutaneous Non-Hodgkin Lymphoma

A B-or T-cell non-Hodgkin lymphoma arising in the skin.


Lugano Classification Stage II Adult Non-Hodgkin Lymphoma AJCC v8

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm. (from AJCC 8th Ed.)


Lung Intravascular Large B-Cell Lymphoma

An aggressive extranodal B-cell non-Hodgkin lymphoma that affects the lung.  It is characterized by the presence of large neoplastic lymphocytes exclusively in the lumina of small vessels, particularly capillaries.


Transformed Mycosis Fungoides

Histologic transformation of mycosis fungoides to an aggressive non-Hodgkin lymphoma.


Adult Lymphoblastic Lymphoma

A lymphoblastic lymphoma occurring in adults.


Mycosis fungoides/Sezary syndrome stage I

Stage I includes: IA (T1, N0, M0, B0-1); IB (T2, N0, M0, B0-1). T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface.  May further stratify into T1a (patch only) vs. T1b (plaque +/- patch).  T2: Patches, papules, or plaques covering 10% or more of the skin surface.  May further stratify into T2a (patch only) vs. T2b (plaque +/- patch).  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  M0: No visceral organ involvement.  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2.  (AJCC 7th ed.)


Refractory High Grade B-Cell Lymphoma with MYC, BCL2, and BCL6 Rearrangements

High grade B-cell lymphoma with MYC, BCL2, and BCL6 rearrangements that is resistant to treatment.


Ann Arbor Stage IV Grade 2 Follicular Lymphoma


grade 3A follicular lymphoma

A grade 3 follicular lymphoma in which centrocytes are present.


Refractory Grade 1 Follicular Lymphoma

Grade 1 follicular lymphoma that is resistant to treatment.


Refractory Primary Vitreoretinal Non-Hodgkin Lymphoma

Primary vitreoretinal non-Hodgkin lymphoma that does not respond to treatment.


Recurrent Adult Immunoblastic Lymphoma

The reemergence of adult immunoblastic lymphoma after a period of remission.


Follicle center lymphoma, follicular grade I, II, III stage IV


Childhood Primary Cutaneous Anaplastic Large Cell Lymphoma

An anaplastic large cell lymphoma that occurs during childhood and is limited to the skin at the time of diagnosis.


Follicular lymphoma stage I

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Colorectal High Grade B-Cell Lymphoma, Not Otherwise Specified

A colorectal high grade B-cell lymphoma with blastoid features or features between diffuse large B-cell lymphoma and Burkitt lymphoma which lacks MYC, BCL2, and BCL6 rearrangements.


Lymphoplasmacytic lymphoma resistant to treatment.


Indolent T-Cell Non-Hodgkin Lymphoma

A T-cell non-Hodgkin lymphoma with an indolent clinical course.


Precursor B-lymphoblastic lymphoma stage II

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


non-Hodgkin's lymphoma of spleen

Non-Hodgkin lymphoma involving the spleen.


Primary Ciliary Body Non-Hodgkin Lymphoma

An extremely rare low-grade B-cell non-Hodgkin lymphoma that arises from the ciliary body. It is characterized by the presence of a diffuse infiltrate of small, round lymphocytes. Most cases represent extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue.


Ann Arbor Stage II Grade 3 Follicular Lymphoma


Cutaneous Nasal Type Extranodal NK/T-Cell Lymphoma

Nasal type extranodal NK/T-cell lymphoma affecting the skin.


Methotrexate-Associated Diffuse Large B-Cell Lymphoma

A diffuse large B-cell lymphoma that develops in patients who are immunosuppressed with methotrexate.


Stage IIIA Mycosis Fungoides and Sezary Syndrome AJCC v7

Stage IIIA includes: T4, N0-2, M0, B0.  T4: Confluence of erythema covering 80% or more of body surface area.  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0-2.  N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3.  M0: No visceral organ involvement.  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  (AJCC 7th ed.)


Recurrent Primary Cutaneous Anaplastic Large Cell Lymphoma

The reemergence of primary cutaneous anaplastic large cell lymphoma after a period of remission.


Tonsillar Diffuse Large B-Cell Lymphoma

Diffuse large B-cell lymphoma that affects the tonsil and the bulk of the tumor is in this anatomic area.


Stage IVA Mycosis Fungoides and Sezary Syndrome AJCC v7

Stage IVA includes: IVA1 (T1-4, N0-2, M0, B2); IVA2 (T1-4, N3, M0, B0-2). T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface.  May further stratify into T1a (patch only) vs. T1b (plaque +/- patch).  T2: Patches, papules, or plaques covering 10% or more of the skin surface.  May further stratify into T2a (patch only) vs. T2b (plaque +/- patch).  T3: One or more tumors (equal or greater than 1 cm diameter).  T4: Confluence of erythema covering 80% or more of body surface area.  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0-2.  N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3.  N3: Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3-4 or NCI LN4; clone positive or negative.  M0: No visceral organ involvement.  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2.  B2: High blood tumor burden: 1000/microL Sezary cells or more with positive clone.  (AJCC 7th ed.)


Burkitt's lymphoma refractory

Burkitt lymphoma that is resistant to treatment.


Extranodal Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma that arises from an anatomic site other than a lymph node.


T-cell childhood lymphoblastic lymphoma

A T lymphoblastic lymphoma that occurs during childhood.


Mycosis fungoides/Sezary syndrome

Mature T and NK neoplasms predominantly affecting the skin and the peripheral blood.  Peripheral blood involvement by abnormal T-cells (cerebriform cells) is a late manifestation of mycosis fungoides, whereas it is the presenting finding in Sezary syndrome.


Localized Anaplastic Large Cell Lymphoma

Anaplastic large cell lymphoma that is confined to a specific site without evidence of spread to other anatomic sites.


Conjunctival Diffuse Large B-Cell Lymphoma

A diffuse large B-cell lymphoma that arises from the conjunctiva.


B-Cell Non-Hodgkin Lymphoma by Ann Arbor Stage

An anatomic stage for B-cell non-Hodgkin lymphoma based on the Ann Arbor classification criteria.


non-Hodgkin's lymphoma of accessory sinus

A non-Hodgkin lymphoma that arises from the paranasal sinus.  Representative examples include diffuse large B-cell lymphoma and extranodal NK/T-cell lymphoma, nasal type.


Cecum Non-Hodgkin Lymphoma

An extranodal non-Hodgkin lymphoma that arises from the cecum.  The majority are B-cell non-Hodgkin lymphomas.


Cutaneous Follicular Lymphoma

A follicular lymphoma involving the skin.  A cutaneous follicular lymphoma may be metastatic to the skin from the lymph nodes or other anatomic sites or primary (cutaneous follicle center lymphoma).


Stage III Childhood Non-Hodgkin Lymphoma

Ann Arbor Classification: Stage III: Involvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE,S).


systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood

Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease of childhood is a rare and very aggressive neoplastic disease emerging after a primary acute or chronic active EBV infection. It presents with persisting fever and malaise, hepatosplenomegaly with or without lymphadenopathy, liver failure, severe pancytopenia and a rapid progression towards multi-organ failure and hemophagocytic syndrome with a fatal issue. It is characterized by clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype.


Systemic Anaplastic Large Cell Lymphoma

An anaplastic large cell lymphoma that is not confined to a single anatomic site and involves multiple nodal and/or extranodal sites.


Colorectal Mucosa-Associated Lymphoid Tissue Lymphoma

An extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue that arises from the colon or rectum.


Lacrimal System Diffuse Large B-Cell Lymphoma

A diffuse large B-cell lymphoma that arises from the lacrimal gland or the lacrimal drainage system.


Primary mediastinal large B-cell lymphoma recurrent

The reemergence of primary mediastinal (thymic) large B-cell lymphoma after a period of remission.


Stage II Adult Burkitt Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


Marginal zone lymphoma recurrent

The reemergence of marginal zone lymphoma after a period of remission.


Ann Arbor Stage IIX (Bulky) Diffuse Large B-Cell Lymphoma

Ann Arbor Stage II diffuse large B-cell lymphoma with a mass of nodes with one diameter more than 10cm or a mediastinal mass larger than one third of the transthoracic width. (Cotswolds modification)


Transformed Marginal Zone Lymphoma to Diffuse Large B-Cell Lymphoma

Histologic transformation of a marginal zone lymphoma to an aggressive diffuse large B-cell lymphoma.


Cutaneous Angioimmunoblastic T-Cell Lymphoma

An aggressive mature T-cell non Hodgkin lymphoma involving the skin. It is characterized by the presence of a polymorphic cellular infiltrate which contains neoplastic small to medium sized T-lymphocytes.


Recurrent Grade III Lymphomatoid Granulomatosis


Diffuse follicle center lymphoma

A lymphoma with the morphologic and immunophenotypic features of a follicular lymphoma that shows entirely a diffuse pattern.  It is usually seen in small biopsy specimens and most probably represents a diffuse area of a follicular lymphoma.


Stage I Adult Immunoblastic Lymphoma

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Intestinal T-cell lymphoma refractory

Enteropathy-associated T-cell lymphoma that is resistant to treatment.


Ann Arbor Stage II Non-Contiguous Adult Non-Hodgkin Lymphoma

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript (e.g., II3). (from PDQ)


Refractory Aggressive B-Cell Non-Hodgkin Lymphoma

An aggressive B-cell non-Hodgkin lymphoma that is resistant to treatment.


Recurrent Aggressive T-Cell Non-Hodgkin Lymphoma

The reemergence of an aggressive T-cell non-Hodgkin lymphoma after a period of remission.


Primary Vitreoretinal Diffuse Large B-Cell Lymphoma

An uncommon and potentially fatal diffuse large B-cell lymphoma that involves the uvea, retina, vitreous body, and optic nerve. It is a subset of primary central nervous system diffuse large B-cell lymphoma.


Stage II Non-Hodgkin Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE). The number of regions involved may be indicated by a subscript (e.g., II3).


Primary cutaneous diffuse large cell B-cell lymphoma

A diffuse large B-cell lymphoma that arises from the skin. This category includes primary cutaneous diffuse large B-cell lymphoma, leg type, and primary cutaneous diffuse large B-cell lymphoma, other, which includes primary cutaneous intravascular large B-cell lymphoma and primary cutaneous T-cell/histiocyte-rich large B-cell lymphoma.


B-cell small lymphocytic lymphoma stage I


mantle cell lymphoma of small intestine

A mantle cell lymphoma that arises from the small intestine.  It may present as an isolated mass in the small intestine or as a part of the multiple lymphomatous polyposis.


Refractory T Lymphoblastic Lymphoma

T-lymphoblastic lymphoma that does not respond to treatment.


Childhood Systemic Anaplastic Large Cell Lymphoma

A systemic anaplastic large cell lymphoma that occurs during childhood.


Pituitary Gland Non-Hodgkin Lymphoma

A rare non-Hodgkin lymphoma that arises from the pituitary gland. The majority are diffuse large B-cell lymphomas.


Parotid Gland Follicular Lymphoma

A follicular lymphoma that manifests initially in the parotid gland.


Refractory High Grade B-Cell Lymphoma

High grade B-cell lymphoma that is resistant to treatment.


Primary Systemic Anaplastic Large Cell Lymphoma, ALK-Negative

A CD30 (Ki-1)-positive anaplastic large cell lymphoma with cytoplasmic and/or nuclear expression of the ALK (anaplastic lymphoma kinase) protein.  It usually affects both the lymph nodes and extranodal sites (primarily skin, bones, lungs, and soft tissues), and has been associated with a favorable clinical outcome.


Burkitt lymphoma associated with AIDS

A Burkitt lymphoma occurring in HIV-positive patients.


MCD diffuse large B-cell lymphoma

A diffuse large B-cell lymphoma that is categorized as MCD with high probability by the LymphGen algorithm. This is based on a combination of genetic features and MCD DLBCLs often, but do not always, have the most common hot spot mutation in MYD88 (L265P) and/or activating mutations in CD79B. This subgroup also commonly has mutations due to aberrant somatic hypermutation affecting PIM1 and/or ETV6, which can be coding or non-coding.


Childhood Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma

A mature T- and NK-cell non-Hodgkin lymphoma that occurs during childhood.


primary cutaneous diffuse large B-cell lymphoma, Leg type

An aggressive primary cutaneous B-cell lymphoma, usually involving the lower leg. It is composed of a generally monotonous proliferation of immunoblasts, or less frequently centroblasts, with few admixed reactive cells. This type of lymphoma occurs most often in elderly women who present with rapidly growing tumors, usually on one or both legs. Dissemination to extracutaneous sites is frequent. Treatment with combination chemotherapy is usually required.


Stage IV Childhood Anaplastic Large Cell Lymphoma

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Chronic Adult T-Cell Leukemia/Lymphoma

An adult T-cell leukemia/ lymphoma associated with skin lesions and mild lymphadenopathy.  Neoplastic lymphocytes are not numerous in the peripheral blood.  Hypercalcemia is absent.


Splenic marginal zone lymphoma recurrent

The reemergence of splenic marginal zone lymphoma after a period of remission.


Mycosis fungoides stage III

Stage III includes: (T4, N0-2, M0, B0-1); IIIA (T4, N0-2, M0, B0); IIIB (T4, N0-2, M0, B1). T4: Confluence of erythema covering 80% or more of body surface area.  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0-2.  N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3.  M0: No visceral organ involvement.  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2.  (AJCC 7th ed.)


Central Nervous System Intravascular Large B-Cell Lymphoma

A rare extranodal B-cell non-Hodgkin lymphoma that affects the central nervous system.  It is characterized by the presence of lymphoma cells exclusively in the lumina of small vessels, particularly capillaries.


Ann Arbor Stage II Non-Contiguous Adult Lymphoblastic Lymphoma

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript (e.g., II3). (from PDQ)


Early Primary Cutaneous T-Cell Non-Hodgkin Lymphoma

Primary cutaneous T-cell non-Hodgkin lymphoma that has spread from its original site of growth to nearby tissues.


Lymphoplasmacytoid lymphoma/immunocytoma stage I

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Stage II Childhood Lymphoblastic Lymphoma

Stage II: Disease extent is limited to a single tumor with regional node involvement, 2 or more tumors or nodal areas involved on one side of the diaphragm, or a primary gastrointestinal tract tumor (completely resected) with or without regional node involvement (from PDQ).


Stage III Adult Immunoblastic Lymphoma

Ann Arbor Classification: Stage III: Involvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE,S).


central nervous system non-hodgkin lymphoma

A non-Hodgkin lymphoma that arises from the central nervous system.


Recurrent Indolent Adult Non-Hodgkin Lymphoma

The reemergence of indolent non-Hodgkin lymphoma in adults after a period of remission.


adult nasal type extranodal NK/T-cell lymphoma

Nasal type extranodal NK/T-cell lymphoma that occurs in adulthood.


Splenic B Lymphoblastic Lymphoma

B-lymphoblastic lymphoma that involves the spleen.


Nodal peripheral T-cell lymphoma with T follicular helper phenotype

A nodal peripheral T-cell non-Hodgkin lymphoma in which the neoplastic lymphocytes manifest a T follicular helper (TFH) cell phenotype. These lymphomas frequently show a diffuse infiltration pattern without a prominent polymorphic inflammatory background, vascular proliferation, or expansion of follicular dendritic cell meshworks. In some cases, a so-called T-zone pattern may be evident. (WHO 2017)


T-cell lymphoma refractory

T-cell non-Hodgkin lymphoma resistant to treatment.


Advanced Primary Cutaneous T-Cell Non-Hodgkin Lymphoma

Primary cutaneous T-cell non-Hodgkin lymphoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


Extranodal marginal zone B-cell lymphoma (MALT type) stage IV

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue presenting as stage IV disease.


Transformed Lymphoplasmacytic Lymphoma to Diffuse Large B-Cell Lymphoma

Histologic transformation of a lymphoplasmacytic lymphoma to an aggressive diffuse large B-cell lymphoma.


Splenic Lymphoblastic Lymphoma

Lymphoblastic lymphoma that affects the spleen.


Ann Arbor Stage I Childhood Non-Hodgkin Lymphoma


Ann Arbor Stage II Contiguous Mantle Cell Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


Stage IIIB Mycosis Fungoides and Sezary Syndrome AJCC v8

Stage IIIB includes: T4, N0-2, M0, B1. T4: Confluence of erythema covering 80% or more of body surface area. N0: No clinically abnormal peripheral lymph nodes; biopsy not required. N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or National Cancer Institute (NCI) LN0-2. N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3. M0: No visceral organ involvement. B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2. (AJCC 8th ed.)


Recurrent Grade 3b Follicular Lymphoma

The reemergence of grade 3b follicular lymphoma after a period of remission.


Stage II Non-Contiguous Adult Indolent Non-Hodgkin Lymphoma

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript (e.g., II3). (from PDQ)


AIDS-Related Lymphoblastic Lymphoma

A lymphoblastic lymphoma occurring in HIV-positive patients.


granulomatous slack skin disease

Granulomatous slack skin (GSS) is a variant of mycosis fungoides (MF), a form of cutaneous T-cell lymphoma, and is characterized by the presence of circumscribed areas of pendulous lax skin.


EBV-Related Non-Hodgkin Lymphoma

Non-Hodgkin lymphoma that is caused by Epstein-Barr virus infection.


Stage I Anaplastic Large Cell Lymphoma

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Centroblastic lymphoma refractory

Centroblastic lymphoma, resistant to treatment.


Stage III Mycosis Fungoides and Sezary Syndrome AJCC v7

Stage III includes: (T4, N0-2, M0, B0-1); IIIA (T4, N0-2, M0, B0); IIIB (T4, N0-2, M0, B1). T4: Confluence of erythema covering 80% or more of body surface area.  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0-2.  N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3.  M0: No visceral organ involvement.  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2.  (AJCC 7th ed.)


Recurrent Immunoblastic Lymphoma

The reemergence of immunoblastic lymphoma after a period of remission.


Leukemic Nonnodal Mantle Cell Lymphoma

Mantle cell lymphoma involving the peripheral blood, bone marrow, and often spleen. It usually has an indolent clinical course.


Aggressive Adult Non-Hodgkin Lymphoma

An aggressive non-Hodgkin lymphoma occurring in adults.  Representative examples include Burkitt lymphoma, precursor B-lymphoblastic lymphoma, precursor T-lymphoblastic lymphoma, and adult T-cell lymphoma/leukemia.


Recurrent Adult Burkitt Lymphoma

The reemergence of adult Burkitt lymphoma after a period of remission.


Advanced Diffuse Large B-Cell Lymphoma

Diffuse large B-cell lymphoma that has spread extensively to distant anatomic sites or is no longer responding to treatment.


Refractory Indolent T-Cell Non-Hodgkin Lymphoma

Indolent T-cell non-Hodgkin lymphoma that is resistant to treatment.


Mycosis Fungoides and Sezary Syndrome by AJCC v8 Stage

A term that refers to the staging of mycosis fungoides and Sezary syndrome, following the rules of the TNM AJCC v8 classification system. This prognostic stage groups system is based on the ISCL/EORTC revision to the staging of mycosis fungoides and Sezary syndrome. There is no prognostic stage groups system for primary cutaneous B-cell/T-cell (non-mycosis fungoides/Sezary syndrome) lymphomas at this time. This staging system does not apply to eyelid skin lymphomas (are staged according to the classification for ocular adnexal lymphoma).  (from AJCC 8th Ed.)


Refractory Transformed B-Cell Non-Hodgkin Lymphoma

An indolent B-cell non-Hodgkin lymphoma which has undergone histologic transformation to an aggressive B-cell non-Hodgkin lymphoma and has become resistant to treatment.


Bladder Mucosa-Associated Lymphoid Tissue Lymphoma

A rare mucosa-associated lymphoid tissue lymphoma affecting the bladder.


Primary mediastinal large B-cell lymphoma refractory

Primary mediastinal (thymic) large B-cell lymphoma that is resistant to treatment.


Burkitt lymphoma in a patient with immunodeficiency.


Recurrent Grade 2 Follicular Lymphoma

The reemergence of grade 2 follicular lymphoma after a period of remission.


B-cell lymphoma stage IV

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Stage IA Mycosis Fungoides and Sezary Syndrome AJCC v8

Stage IA includes: T1, N0, M0, B0,1. T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface. N0: No clinically abnormal peripheral lymph nodes; biopsy not required. M0: No visceral organ involvement. B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells). B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2. (AJCC 8th ed.)


Pediatric nodal marginal zone lymphoma

A nodal marginal zone lymphoma affecting the pediatric population, predominantly males.  It presents with asymptomatic and localized (mainly head and neck) disease in the vast majority of cases.  Morphologically there is often progressive transformation of the germinal centers.  The prognosis is excellent.


Diffuse Large B-Cell Lymphoma, Not Otherwise Specified by Gene Expression Profile

A header term that includes the following diffuse large B-cell lymphoma subtypes by gene expression profiling: activated B-cell-like diffuse large B-cell lymphoma, germinal center B-cell-like diffuse large B-cell lymphoma, and type 3 diffuse large B-cell lymphoma.


Recurrent Childhood Grade III Lymphomatoid Granulomatosis

The reemergence of grade III lymphomatoid granulomatosis in childhood after a period of remission.


primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma is a rare form of primary cutaneous T-cell lymphoma characterized by rapidly progressing, localized or disseminated nodules, tumors or eczematous skin lesions. It has a particularly aggressive clinical course with a high tendency to spread, in advanced stages, to extracutaneous locations (the central nervous system, lung, testes). Lymph nodes are often spared.


Refractory Intravascular Large B-Cell Lymphoma

Intravascular large B-cell lymphoma that is resistant to treatment.


heavy chain disease

Heavy-chain diseases (HCDs) are rare monoclonal lymphoplasma-cell proliferative disorders involving B cells and are characterized by the synthesis of truncated heavy chains without associated light chains.


Refractory Indolent Non-Hodgkin Lymphoma

Indolent non-Hodgkin lymphoma that is resistant to treatment.


Ann Arbor Stage IV Grade 1 Follicular Lymphoma


Pleural Primary Effusion Lymphoma

Primary effusion lymphoma that affects the pleural cavity. The majority of patients are HIV-positive men.


Favorable Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma with favorable prognosis.


ocular adnexal lymphoma

A non-Hodgkin lymphoma arising from the conjunctiva, lacrimal gland, lacrimal drainage apparatus, eyelids, or other orbital tissues around the eye. The vast majority of cases are extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue, however, other histologic types of lymphomas can originate from ocular adnexal tissues, including rare cases of NK/T-cell lymphomas of nasal type.


Ann Arbor Stage II Non-Contiguous Adult Burkitt Lymphoma

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript (e.g., II3). (from PDQ)


Salivary Gland Mucosa-Associated Lymphoid Tissue Lymphoma

A low-grade extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue arising from a salivary gland. It usually occurs in the parotid gland and is often associated with Sjogren syndrome. The prognosis is favorable.


high grade B-cell lymphoma

A term that refers to high grade B-cell lymphoma, not otherwise specified or high grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements.


Ann Arbor Stage I Grade 3 Follicular Lymphoma

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Recurrent Subcutaneous Panniculitis-Like T-Cell Lymphoma

The reemergence of subcutaneous panniculitis-like T-cell lymphoma after a period of remission.


retina lymphoma

A lymphoma that involves the retina.


Refractory NK-Cell Lymphoma, Unclassifiable

NK-cell lymphoma, unclassifiable that is resistant to treatment.


Refractory Follicular T-Cell Lymphoma

Follicular T-cell lymphoma that is resistant to treatment.


Pancreatic Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma that arises from the pancreas with the bulk of the tumor localized to this organ.  The vast majority of cases are of B-cell phenotype and include mucosa-associated lymphoid tissue lymphomas, follicular lymphomas, and diffuse large B-cell lymphomas.


Precursor B-lymphoblastic lymphoma stage IV

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Body Cavity Primary Effusion Lymphoma

A large B-cell lymphoma presenting as a serous effusion without detectable tumor masses. It is universally associated with human herpes virus 8 (HHV8), also called Kaposi sarcoma-associated herpesvirus.  It mostly occurs in the setting of immunodeficiency. The most common sites of involvement are the pleural, pericardial, and peritoneal cavities. (WHO 2017)


Stage II Adult Immunoblastic Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE). The number of regions involved may be indicated by a subscript (e.g., II3).


Stage IA Mycosis Fungoides AJCC v7

Stage IA includes: T1, N0, M0, B0-1.  T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface.  May further stratify into T1a (patch only) vs. T1b (plaque +/- patch).  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  M0: No visceral organ involvement.  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2.  (AJCC 7th ed.)


Small Intestinal T-Cell Non-Hodgkin Lymphoma

A T-cell non-Hodgkin lymphoma that arises from the small intestine.  It includes the enteropathy-associated T-cell lymphoma and the monomorphic CD56 positive intestinal T-cell lymphoma.


Stage II Contiguous Adult Indolent Non-Hodgkin Lymphoma


Angiocentric lymphoma refractory

Nasal type extranodal NK/T-cell lymphoma that does not respond to treatment.


AIDS-Related Plasmablastic Lymphoma

A plasmablastic lymphoma in a patient with acquired immunodeficiency syndrome.


High Grade B-Cell Lymphoma, Not Otherwise Specified

A high grade B-cell lymphoma with blastoid features or features between diffuse large B-cell lymphoma and Burkitt lymphoma which lacks MYC, BCL2, and BCL6 rearrangements.


Pediatric follicular lymphoma

A nodal follicular lymphoma with favorable prognosis. It lacks BCL-2 rearrangement and 14;18 translocation. Nearly all cases are localized. It is usually seen in the pediatric population but similar lymphomas may occur in adults.


Sinonasal Extranodal NK/T-Cell Lymphoma

An extranodal NK/T-cell lymphoma that arises from the sinonasal tract.


large B-cell diffuse lymphoma of kidney

Diffuse large B-cell lymphoma that affects the kidney.


Diffuse Large B-Cell Lymphoma Post-Transplant Lymphoproliferative Disorder

A diffuse large B-cell lymphoma that arises in a patient with a history of organ transplantation.


Lymphoma, Mixed-Cell, Follicular

A follicular lymphoma which contains 6-15 centroblasts per 40X high-power microscopic field.


EZB diffuse large B-cell lymphoma

A diffuse large B-cell lymphoma that is categorized as EZB with high probability by the LymphGen algorithm. This is based on a combination of genetic features and EZB DLBCLs often, but do not always, have hot spot mutations in EZH2 and/or a BCL2 translocation. This class can be further subdivided into two sub-classes EZB-MYC+ and EZB-MYC- using the double hit gene expression signature (DHITsig). This subgroup also commonly has mutations due to aberrant somatic hypermutation affecting IRF8, which can be coding or non-coding.


B-cell lymphoma stage III

Ann Arbor Classification: Stage III: Involvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE,S).


Anaplastic Large Cell Lymphoma, Giant Cell Rich Subtype

An anaplastic large cell lymphoma characterized by the presence of numerous giant cells.


Cutaneous Mantle Cell Lymphoma

Mantle cell lymphoma that affects the skin.


Ann Arbor Stage II Grade 2 Follicular Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


Recurrent Diffuse Large B-Cell Lymphoma, Not Otherwise Specified

The reemergence of diffuse large B-cell lymphoma, not otherwise specified after a period of remission.


Recurrent T Lymphoblastic Lymphoma

The reemergence of T-lymphoblastic lymphoma after a period of remission.


Centroblastic Lymphoma Post-Transplant Lymphoproliferative Disorder

A centroblastic lymphoma that arises in a patient with a history of organ transplantation.


Refractory Transformed Mycosis Fungoides

Transformed mycosis fungoides that is resistant to treatment.


B-cell small lymphocytic lymphoma stage IV

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Lacrimal System Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma that arises from the lacrimal gland or the lacrimal drainage system. The majority of cases are of B-cell type. The most common subtypes are mucosa-associated lymphoid tissue lymphoma, follicular lymphoma, and diffuse large B-cell lymphoma.


Childhood Non-Hodgkin Lymphoma

Non-Hodgkin lymphoma occurring in childhood.


Stage I Non-Hodgkin Lymphoma

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Small Lymphocytic Lymphoma with Plasmacytoid Differentiation

A small lymphocytic lymphoma characterized by the presence of neoplastic lymphocytes with plasmacytoid morphology.


Intestinal T-Cell Lymphoma

A group of mature T-cell and NK-cell non-Hodgkin lymphomas that includes enteropathy-associated T-cell lymphoma, monomorphic epitheliotropic intestinal T-cell lymphoma, and intestinal T-cell lymphoma, not otherwise specified. Most of these lymphomas arise from the small intestine and a minority from the large intestine or stomach.


Duodenal-type follicular lymphoma

A localized low-grade follicular lymphoma within the gastrointestinal tract, which is distinct from other gastrointestinal tract follicular lymphomas.  It has features that overlap with in situ follicular neoplasia as well as some features resembling an extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. The prognosis is excellent.


Lymphoplasmacytoid lymphoma/immunocytoma stage IV


Oropharyngeal Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma that arises from the oropharynx.


Stage I Indolent Adult Non-Hodgkin Lymphoma


Lugano Classification Stage II Bulky Adult Non-Hodgkin Lymphoma AJCC v8

Stage II Bulky: Stage II with disease bulk. (from AJCC 8th Ed.)


Lymphomatous Adult T-Cell Leukemia/Lymphoma

An adult T-cell leukemia/lymphoma characterized by generalized lymphadenopathy without peripheral blood involvement.  Most patients have advanced disease, however hypercalcemia is not frequently present.


Pleomorphic Variant Mantle Cell Lymphoma

An aggressive mantle cell lymphoma characterized by the presence of pleomorphic neoplastic B-lymphocytes.


Burkitt's lymphoma of spleen

Burkitt lymphoma that involves the spleen.


Peripheral T-Cell Lymphoma, Unclassifiable

A very rare peripheral T-cell lymphoma with pathologic and clinical features posing difficulty in its exact diagnosis and classification.


Major Salivary Gland Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma that arises from the parotid gland, submandibular gland, or sublingual gland.  Representative examples include mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma.


Ann Arbor Stage II Grade 3 Non-Contiguous Follicular Lymphoma

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript (e.g., II3). (from PDQ)


Lymphoplasmacytoid lymphoma/immunocytoma stage II

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


Recurrent NK-Cell Lymphoma, Unclassifiable

The reemergence of NK-cell lymphoma, unclassifiable after a period of remission.


Diffuse large B-cell lymphoma, unclassifiable, NOS

A biologic subset of diffuse large B-cell lymphomas (DLBCL) that do not overexpress the genes whose overexpression characterizes the germinal center B-cell-like and activated B-cell-like DLBCL.  Morphologically, these lymphomas are either immunoblastic or centroblastic.  Patients with this type of diffuse large B-cell lymphoma are reported to have a less favorable outcome, similar to those with an activated B-cell-like gene expression profile.


Recurrent Follicular T-Cell Lymphoma

The reemergence of follicular T-cell lymphoma after a period of remission.


Stage IVA2 Mycosis Fungoides and Sezary Syndrome AJCC v8

Stage IVA2 includes: T1-4, N3, M0, B0-2. T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface. T2: Patches, papules, or plaques covering 10% or more of the skin surface. T3: One or more tumors (equal or greater than 1 cm in diameter). T4: Confluence of erythema covering 80% or more of body surface area. N3: Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3-4 or NCI LN4; clone positive or negative. M0: No visceral organ involvement. B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells). B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2. B2: High blood tumor burden: 1,000 per microliter or more Sezary cells with positive clone. (AJCC 8th ed.)


mycosis fungoides variant

A header term that includes the rare following variants of mycosis fungoides: folliculotropic mycosis fungoides, granulomatous slack skin disease, and pagetoid reticulosis.


Primary Bone Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma that arises from the bone, without lymph node or other extranodal involvement.  The femur, spine, and pelvic bones are the most frequently affected areas.  The majority of cases are diffuse large B-cell lymphomas.  Most patients present with pain in the affected area.  Systemic symptoms are rare.


Recurrent Primary Vitreoretinal Non-Hodgkin Lymphoma

The reemergence of primary vitreoretinal non-Hodgkin lymphoma after a period of remission.


Stage I Mycosis Fungoides and Sezary Syndrome AJCC v8

Stage I includes: IA (T1, N0, M0, B0,1); IB (T2, N0, M0, B0,1). T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface. T2: Patches, papules, or plaques covering 10% or more of the skin surface. N0: No clinically abnormal peripheral lymph nodes; biopsy not required. M0: No visceral organ involvement. B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells). B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2. (AJCC 8th ed.)


Methotrexate-Associated Peripheral T-Cell Lymphoma

A peripheral T-cell lymphoma that develops in patients who are immunosuppressed with methotrexate.


intravascular large B-cell lymphoma

Intravascular large B-cell lymphoma (IVLBCL) is a very rare form of diffuse large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumina of small blood vessels (especially the capillaries) that most often presents with a wide range of clinical manifestations (as potentially any tissue can be involved), with patients from Western countries more frequently manifesting with neurological and cutaneous symptoms while patients from Asian countries more frequently displaying hepatosplenomegaly and thrombocytopenia. IVLBCL is characterized by an absence of lymphadenopathy, an aggressive clinical course and a poor prognosis.


Refractory Diffuse Large B-Cell Lymphoma, Not Otherwise Specified

Diffuse large B-cell lymphoma, not otherwise specified that is resistant to treatment.


Refractory Lymphoblastic Lymphoma

Lymphoblastic lymphoma that is resistant to treatment.


Ann Arbor Stage II Grade 2 Non-Contiguous Follicular Lymphoma


Refractory Grade 3b Follicular Lymphoma

Grade 3b follicular lymphoma that is resistant to treatment.


Stage IV Adult Lymphoblastic Lymphoma

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Small Intestinal Monomorphic Epitheliotropic T-Cell Lymphoma

A monomorphic epitheliotropic intestinal T-cell lymphoma arising from the small intestine. It is characterized by the presence of a monomorphic cellular infiltrate of small to medium-sized T-lymphocytes that are cytotoxic and express CD56. It is not associated with celiac disease.


Methotrexate-Related Burkitt Lymphoma

A Burkitt lymphoma that develops in patients who are immunosuppressed with methotrexate.


Ann Arbor Stage II Grade 3 Contiguous Follicular Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


Stage II Mycosis Fungoides and Sezary Syndrome AJCC v8

Stage II includes: IIA (T1,2, N1,2, M0, B0,1); IIB (T3, N0-2, M0, B0,1). T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface. T2: Patches, papules, or plaques covering 10% or more of the skin surface. T3: One or more tumors (equal or greater than 1 cm in diameter). N0: No clinically abnormal peripheral lymph nodes; biopsy not required. N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or National Cancer Institute (NCI) LN0-2. N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3. M0: No visceral organ involvement. B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells). B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2. (AJCC 8th ed.)


Recurrent Extranodal Diffuse Large B-cell Lymphoma

The reemergence of extranodal diffuse large B-cell lymphoma after a period of remission.


Nodal marginal zone B-cell lymphoma stage IV


Cutaneous Burkitt Lymphoma

Burkitt lymphoma that affects the skin.


large B-cell diffuse lymphoma of heart

A diffuse large B-cell lymphoma that arises from the heart.


large B-cell diffuse lymphoma of accessory sinus

A non-Hodgkin lymphoma that arises from the paranasal sinus and is characterized by the presence of a diffuse malignant infiltrate composed of large B-lymphocytes.


Ann Arbor Stage III Grade 2 Follicular Lymphoma

Ann Arbor Classification: Stage III: Involvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE,S).


A follicular lymphoma that develops in patients who are immunosuppressed with methotrexate.


Stage IIA Mycosis Fungoides and Sezary Syndrome AJCC v8

Stage IIA includes:  T1-2, N1,2, M0, B0,1. T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface. T2: Patches, papules, or plaques covering 10% or more of the skin surface. N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or National Cancer Institute (NCI) LN0-2. N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3. M0: No visceral organ involvement. B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells). B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2. (AJCC 8th ed.)


Digestive System Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma that arises from any part of the digestive system, with the bulk of the disease localized to that site.


Ann Arbor Stage II Contiguous Adult Lymphoblastic Lymphoma

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript (e.g., II3). (from PDQ)


Grade 2 Diffuse Follicular Lymphoma

A follicular lymphoma with diffuse pattern characterized by the presence of 6-15 centroblasts per 40 high-power fields.


Non-Hodgkin's lymphoma transformed recurrent

The reemergence of non-Hodgkin lymphoma transformed after a period of remission.


Recurrent Anaplastic Large Cell Lymphoma

The reemergence of anaplastic large cell lymphoma after a period of remission.


Marginal zone lymphoma stage II

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript (e.g., II3). (PDQ)


Esophageal Diffuse Large B-Cell Lymphoma

An extranodal diffuse large B-cell lymphoma that arises from the esophagus with the bulk of the mass located in the esophagus.


Angioimmunoblastic T-cell lymphoma refractory

Angioimmunoblastic T-cell lymphoma resistant to treatment.


Refractory Indolent Adult Non-Hodgkin Lymphoma

Indolent adult non-Hodgkin lymphoma that is resistant to treatment.


Childhood Lymphoblastic Lymphoma

A lymphoblastic lymphoma occurring in children.


Recurrent High Grade B-Cell Lymphoma with MYC and BCL2 or BCL6 Rearrangements

The reemergence of high grade B-cell lymphoma with MYC and BCL2 or BCL6 rearrangements after a period of remission.


Stage IVB Mycosis Fungoides and Sezary Syndrome AJCC v8

Stage IVB includes: T1-4, N0-3, M1, B0-2. T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface. T2: Patches, papules, or plaques covering 10% or more of the skin surface. T3: One or more tumors (equal or greater than 1 cm in diameter). T4: Confluence of erythema covering 80% or more of body surface area. N0: No clinically abnormal peripheral lymph nodes; biopsy not required. N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or National Cancer Institute (NCI) LN0-2. N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3. N3: Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3-4 or NCI LN4; clone positive or negative. M1: Visceral involvement (must have pathology confirmation and organ involved should be specified). B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells). B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2. B2: High blood tumor burden: 1,000 per microliter or more Sezary cells with positive clone. (AJCC 8th ed.)


Advanced Unfavorable Non-Hodgkin Lymphoma

A clinical stage II or IV non-Hodgkin lymphoma with at least four of the following risk factors: albumin level less than 4.0 g/dl, hemoglobin level of less than 10.5 g/dl, male sex, forty-five years of age or older, stage IV disease, white cell count of at least 15,000 per cubic millimeter, absolute lymphocytic count of less than 600 per cubic millimeter or a lymphocytic count of less than 8% of the total white cell count.


Recurrent High Grade B-Cell Lymphoma with MYC, BCL2, and BCL6 Rearrangements

The reemergence of high grade B-cell lymphoma with MYC, BCL2, and BCL6 rearrangements after a period of remission.


Cutaneous Adult T-Cell Leukemia/Lymphoma

Adult T-cell leukemia/lymphoma affecting the skin.


Recurrent Plasmablastic Lymphoma

The reemergence of plasmablastic lymphoma after a period of remission.


primary mediastinal large B-cell lymphoma

A large B-cell non-Hodgkin lymphoma arising in the mediastinum. Morphologically it is characterized by a massive diffuse lymphocytic proliferation associated with compartmentalizing fibrosis. Response to intensive chemotherapy, with or without radiotherapy, is usually good. (WHO, 2001)


Stage I Childhood Anaplastic Large Cell Lymphoma

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Endometrial Mucosa-Associated Lymphoid Tissue Lymphoma

A rare extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue affecting the endometrium.


NK-Cell Lymphoma, Unclassifiable

A very rare NK-cell lymphoma with pathologic and clinical features posing difficulty in its exact diagnosis and classification.


Central Nervous System Anaplastic Large Cell Lymphoma, ALK-Positive

An anaplastic large cell lymphoma, ALK-positive, arising from the central nervous system.


large B-cell diffuse lymphoma of nasal cavity

A non-Hodgkin lymphoma that arises from the nasal cavity and is characterized by the presence of a diffuse malignant infiltrate composed of large B-lymphocytes.


St. Jude Stage IV Childhood Non-Hodgkin Lymphoma AJCC v8

Stage IV: Any tumor, with initial CNS and/or bone marrow involvement. (from AJCC 8th Ed.)


St. Jude Childhood Non-Hodgkin Lymphoma by AJCC v8 Stage

A staging system for childhood non-Hodgkin Lymphomas based on the St. Jude Children's Research Hospital staging system. The Ann Arbor staging system has been found to be inappropriate for staging of the non-Hodgkin lymphomas of childhood. The St. Jude staging system has been widely accepted for more than three decades and remains the recommended staging system for childhood non-Hodgkin lymphomas. (from AJCC 8th Ed.)


Marginal zone lymphoma stage IV

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Recurrent Adult Grade III Lymphomatoid Granulomatosis

The reemergence of grade III lymphomatoid granulomatosis in adulthood after a period of remission.


Recurrent Transformed Indolent B-Cell Non-Hodgkin Lymphoma to Diffuse Large B-Cell Lymphoma

The reemergence of diffuse large B-cell lymphoma that resulted from the transformation of indolent B-cell non-Hodgkin lymphoma, after a period of remission.


Refractory Extranodal Diffuse Large B-cell Lymphoma

Extranodal diffuse large B-cell lymphoma that is resistant to treatment.


Ann Arbor Stage II Childhood Non-Hodgkin Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


Refractory Transformed Indolent B-Cell Non-Hodgkin Lymphoma to Diffuse Large B-Cell Lymphoma

Diffuse large B-cell lymphoma that resulted from the transformation of indolent B-cell non-Hodgkin lymphoma and is resistant to treatment.


Refractory Monomorphic Epitheliotropic Intestinal T-cell Lymphoma

Monomorphic epitheliotropic intestinal T-cell lymphoma that is resistant to treatment.


Adult Grade III Lymphomatoid Granulomatosis

Grade III lymphomatoid granulomatosis that occurs in adulthood.


Non-Hodgkin Lymphoma with Variable Clinical Course

A non-Hodgkin lymphoma with variable clinical course.


Recurrent Aggressive B-Cell Non-Hodgkin Lymphoma

The reemergence of an aggressive B-cell non-Hodgkin lymphoma after a period of remission.


Peritoneal Primary Effusion Lymphoma

Primary effusion lymphoma that affects the peritoneal cavity. The majority of patients are HIV-positive men.


T-Cell Non-Hodgkin Lymphoma by Ann Arbor Stage

An anatomic stage for T-cell non-Hodgkin lymphoma based on the Ann Arbor classification criteria.


Sezary syndrome

Sezary syndrome (SS) is an aggressive form of cutaneous T-cell lymphoma characterized by a triad of erythroderma, lymphadenopathy and circulating atypical lymphocytes (Sezary cells).


Secondary Choroidal Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma that has spread to the choroid following the initial presentation in another nodal or extranodal site.


Stage III Burkitt Lymphoma

Ann Arbor Classification: Stage III: Involvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE,S).


Non-Hodgkin's lymphoma of prostate

A rare non-Hodgkin lymphoma that arises from the prostate gland.


Lymphoma, Small Cleaved-Cell, Follicular

A follicular lymphoma which contains up to 5 centroblasts per 40X high-power microscopic field.


Noncutaneous Childhood Anaplastic Large Cell Lymphoma by Ann Arbor Stage

An anatomic stage for noncutaneous childhood anaplastic large cell lymphoma based on the Ann Arbor classification criteria.


Advanced Favorable Non-Hodgkin Lymphoma

A clinical stage II or IV non-Hodgkin lymphoma with up to three of the following risk factors: albumin level less than 4.0 g/dl, hemoglobin level of less than 10.5 g/dl, male sex, forty-five years of age or older, stage IV disease, white cell count of at least 15,000 per cubic millimeter, absolute lymphocytic count of less than 600 per cubic millimeter or a lymphocytic count of less than 8% of the total white cell count.


Large B-cell lymphoma with IRF4 rearrangement

A large B-cell lymphoma typically affecting the Waldeyer ring and/or cervical lymph nodes. It occurs most commonly in children and young adults and is low stage. It is characterized by a follicular, follicular and diffuse, or pure diffuse growth pattern resembling follicular lymphoma grade 3B or a diffuse large B-cell lymphoma. Most cases have IG/IRF4 rearrangements. BCL2 rearrangements are not present. Some cases that belong in this category lack an IRF4 rearrangement but have strong IRF4/MUM1 expression. This lymphoma is more aggressive than pediatric-type follicular lymphomas.


Angiocentric lymphoma recurrent

The reemergence of nasal type extranodal NK/T-cell lymphoma after a period of remission.


Stage IV Anaplastic Large Cell Lymphoma

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Refractory Immunoblastic Lymphoma

Immunoblastic lymphoma that is resistant to treatment.


Transformed Follicular Lymphoma to Diffuse Large B-Cell Lymphoma

Histologic transformation of a follicular lymphoma to an aggressive diffuse large B-cell lymphoma.


Classical Burkitt Lymphoma

A Burkitt lymphoma characterized by the presence of a uniform malignant lymphocytic infiltrate that is composed of medium-sized cells with round nuclei and multiple basophilic nucleoli, abundant mitotic figures, and a starry-sky pattern due to the presence of multiple tangible body macrophages.


Precursor B-lymphoblastic lymphoma stage I

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Splenic marginal zone lymphoma refractory

Splenic marginal zone lymphoma that is resistant to treatment.


N1 diffuse large B-cell lymphoma

A diffuse large B-cell lymphoma that is categorized as N1 with high probability by the LymphGen algorithm. This is based on a combination of genetic features. Although N1 DLBCLs always have an activating mutation affecting NOTCH1, LymphGen can assign cases with this mutation to other classes, depending on the presence of other genetic features.


Immunoblastic lymphoma associated with AIDS

A morphologic variant of diffuse large B-cell lymphoma occurring in HIV-positive patients.


Refractory Follicular Lymphoma

Follicular lymphoma grade I, II, III that is resistant to treatment.


Recurrent Follicular Lymphoma

The reemergence of follicular lymphoma after a period of remission.


lymphoplasmacytic lymphoma

A clonal neoplasm of small B-lymphocytes, lymphoplasmacytoid cells, and plasma cells involving the bone marrow, lymph nodes, and the spleen. The majority of patients have a serum IgM paraprotein.


T-cell lymphoma recurrent

The reemergence of T-cell non-Hodgkin lymphoma after a period of remission.


Lugano Classification Adult Non-Hodgkin Lymphoma by AJCC v8 Stage

A staging system for adult non-Hodgkin lymphomas based on the Lugano classification criteria. This staging system does not apply to ocular adnexal lymphomas (are staged according to the classification for ocular adnexal lymphoma), pediatric non-Hodgkin lymphomas (are staged according to the St. Jude Children's Research Hospital staging system), primary cutaneous lymphomas (are staged according to the classification for primary cutaneous lymphoma), and multiple myelomas (are staged according to the classification for plasma cell myeloma). (from AJCC 8th Ed.)


Stage IV Mycosis Fungoides and Sezary Syndrome AJCC v8

Stage IV includes: IVA1 (T1-4, N0-2, M0, B2); IVA2 (T1-4, N3, M0, B0-2); IVB (T1-4, N0-3, M1, B0-2). T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface. T2: Patches, papules, or plaques covering 10% or more of the skin surface. T3: One or more tumors (equal or greater than 1 cm in diameter). T4: Confluence of erythema covering 80% or more of body surface area. N0: No clinically abnormal peripheral lymph nodes; biopsy not required. N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or National Cancer Institute (NCI) LN0-2. N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3. N3: Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3-4 or NCI LN4; clone positive or negative. M0: No visceral organ involvement.  M1: Visceral involvement (must have pathology confirmation and organ involved should be specified). B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells). B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2. B2: High blood tumor burden: 1,000 per microliter or more Sezary cells with positive clone. (AJCC 8th ed.)


Lacrimal Drainage System Diffuse Large B-Cell Lymphoma

A diffuse large B-cell lymphoma that arises from the lacrimal sac.


aggressive B-cell non-Hodgkin lymphoma

A B-cell non-Hodgkin lymphoma with an aggressive clinical course. This category includes B lymphoblastic lymphoma, subtypes of diffuse large B-cell lymphoma, Burkitt lymphoma, grade 3 follicular lymphoma, and mantle cell lymphoma.


Recurrent Intravascular Large B-Cell Lymphoma

The reemergence of intravascular large B-cell lymphoma after a period of remission.


Stage III Adult Lymphoblastic Lymphoma

Ann Arbor Classification: Stage III: Involvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE,S).


Mycosis fungoides/Sezary syndrome stage IV

Stage IV includes: IVA1 (T1-4, N0-2, M0, B2); IVA2 (T1-4, N3, M0, B0-2); IVB (T1-4, N0-3, M1, B0-2). T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface.  May further stratify into T1a (patch only) vs. T1b (plaque +/- patch).  T2: Patches, papules, or plaques covering 10% or more of the skin surface.  May further stratify into T2a (patch only) vs. T2b (plaque +/- patch).  T3: One or more tumors (equal or greater than 1 cm diameter).  T4: Confluence of erythema covering 80% or more of body surface area.  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0-2.  N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3.  N3: Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3-4 or NCI LN4; clone positive or negative.  M0: No visceral organ involvement.  M1: Visceral involvement (must have pathology confirmation and organ involved should be specified).  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2.  B2: High blood tumor burden: 1000/microL Sezary cells or more with positive clone.  (AJCC 7th ed.)


Parotid Gland Diffuse Large B-Cell Lymphoma

A diffuse large B-cell lymphoma that manifests initially in the parotid gland.


Lugano Classification Stage I Adult Non-Hodgkin Lymphoma AJCC v8

Stage I: Involvement of a single lymphatic site (i.e., nodal region, Waldeyer's ring, thymus, or spleen). (from AJCC 8th Ed.)


Gastric T-Cell Non-Hodgkin Lymphoma

A rare, extranodal T-cell non-Hodgkin lymphoma that arises from the stomach with the bulk of the mass located in the stomach.


Advanced Marginal Zone Lymphoma

Marginal zone lymphoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


childhood nasal type extranodal NK/T-cell lymphoma

A nasal type extranodal NK/T-cell lymphoma occurring in childhood.


Advanced Transformed Non-Hodgkin Lymphoma

Transformed non-Hodgkin lymphoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


Primary Cutaneous Intravascular Large B-Cell Lymphoma

Intravascular large B-cell lymphoma that arises from the skin.


B-cell lymphoma stage I

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Recurrent Peripheral T-Cell Lymphoma, Not Otherwise Specified

The reemergence of peripheral T-cell lymphoma, not otherwise specified after a period of remission.


Nodal marginal zone B-cell lymphoma recurrent

The reemergence of nodal marginal zone lymphoma after a period of remission.


Campylobacter Jejuni-Associated Small Intestinal Mucosa-Associated Lymphoid Tissue Lymphoma

A mucosa-associated lymphoid tissue lymphoma that arises from the small intestine and it is associated with Campylobacter jejuni infection.


Centroblastic lymphoma stage IV

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Esophageal Mucosa-Associated Lymphoid Tissue Lymphoma

An extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue that arises from the esophagus with the bulk of the mass located in the esophagus.


Recurrent Grade 3a Follicular Lymphoma

The reemergence of grade 3a follicular lymphoma after a period of remission.


Sporadic Burkitt's lymphoma

A clinical variant of Burkitt lymphoma that occurs throughout the world.  It affects both children and adults and is more frequently seen in males.


Extranodal marginal zone B-cell lymphoma (MALT type) stage I

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue presenting as stage I disease.


Adult T-cell lymphoma/leukemia stage II

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


Pituitary Gland Diffuse Large B-Cell Lymphoma

A rare diffuse large B-cell lymphoma that arises from the pituitary gland.


Recurrent Childhood Anaplastic Large Cell Lymphoma

The reemergence of anaplastic large cell lymphoma in childhood after a period of remission.


Precursor B-lymphoblastic lymphoma refractory

B-lymphoblastic lymphoma that does not respond to treatment.


Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma

An extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue that arises from the ocular adnexa. It is the most common type of primary ocular adnexal lymphoma.


Anaplastic large B-cell lymphoma

A diffuse large B-cell lymphoma variant characterized by the presence of large round, oval, or polygonal cells with bizarre pleomorphic nuclei resembling Hodgkin or Reed-Sternberg cells. It is unrelated to anaplastic large cell lymphoma which is a T-cell non-Hodgkin lymphoma.


Stage III Childhood Lymphoblastic Lymphoma

Stage III: Tumors or involved lymph node areas occur on both sides of the diaphragm. Stage III also includes any primary intrathoracic (mediastinal, pleural, or thymic) disease, extensive primary intra-abdominal disease, or any paraspinal or epidural tumors (from PDQ).


Dural Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue

A rare, primary marginal zone lymphoma of mucosa-associated lymphoid tissue that arises from the meningeal dura.  The majority of cases have been described in middle aged women.  Radiologically, the lymphomatous dural infiltration mimics meningioma.


Recurrent Indolent Non-Hodgkin Lymphoma

The reemergence of indolent non-Hodgkin lymphoma after a period of remission.


Adult Immunoblastic Lymphoma

A morphologic variant of diffuse large B-cell lymphoma occurring in adults.  It is characterized by the presence of large lymphoid cells with abundant cytoplasm and prominent nucleoli.


Stage II Adult Lymphoblastic Lymphoma

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript (e.g., II3). (from PDQ)


Transformed Small Lymphocytic Lymphoma to Diffuse Large B-Cell Lymphoma

Histologic transformation of a small lymphocytic lymphoma to an aggressive diffuse large B-cell lymphoma.


B Lymphoblastic Lymphoma by Ann Arbor Stage

An anatomic stage for B-lymphoblastic lymphoma based on the Ann Arbor classification criteria.


Stage II Childhood Anaplastic Large Cell Lymphoma


Refractory Primary Vitreoretinal Diffuse Large B-Cell Lymphoma

Primary vitreoretinal diffuse large B-cell lymphoma that does not respond to treatment.


intraocular lymphoma

A lymphoma that arises within the eye. Signs and symptoms include decreased vision, uveitis, and vitreous floaters.


Stage IIIB Mycosis Fungoides and Sezary Syndrome AJCC v7

Stage IIIB includes: T4, N0-2, M0, B1. T4: Confluence of erythema covering 80% or more of body surface area.  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0-2.  N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3.  M0: No visceral organ involvement.  B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2.  (AJCC 7th ed.)


Recurrent Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System

The reemergence of primary diffuse large B-cell lymphoma of the central nervous system after a period of remission.


Ann Arbor Stage II Non-Contiguous Aggressive Adult Non-Hodgkin Lymphoma


Childhood Diffuse Large B-Cell Lymphoma

A diffuse large B-cell lymphoma that occurs during childhood.


Parotid Gland Mantle Cell Lymphoma

A mantle cell lymphoma that manifests initially in the parotid gland.


subcutaneous panniculitis-like T-cell lymphoma

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic cutaneous lymphoma that has been recognized as a distinct subset of peripheral T-cell lymphomas originating and presenting primarily in the subcutaneous fat tissue.


Recurrent Childhood Non-Hodgkin Lymphoma

The reemergence of non-Hodgkin lymphoma in childhood after a period of remission.


Refractory Central Nervous System Non-Hodgkin Lymphoma

A central nervous system non-Hodgkin lymphoma which does not respond to treatment.


Stage II Burkitt Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


Refractory Hepatosplenic T-Cell Lymphoma

Hepatosplenic T-cell lymphoma that is resistant to treatment.


HHV8-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified

A large B-cell lymphoma characterized by the presence of a diffuse proliferation of large, atypical HHV8-positive neoplastic lymphocytes. It usually arises in association with HHV8-positive multicentric Castleman disease.


Childhood Burkitt Lymphoma

A Burkitt lymphoma occurring in children.


Ann Arbor Stage IV Aggressive Adult Non-Hodgkin Lymphoma

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Burkitt Lymphoma by Ann Arbor Stage

An anatomic stage for Burkitt lymphoma based on the Ann Arbor classification criteria.


Breast implant-associated anaplastic large cell lymphoma

A rare anaplastic large cell lymphoma that can develop following breast implants. It has been reported in patients with saline-and silicone-filled implants. It usually presents as an accumulation of seroma fluid between the implant and the surrounding fibrous capsule. The median interval from the time of the implant to the development of lymphoma is approximately 10 years.


Colorectal Non-Hodgkin Lymphoma

An extranodal non-Hodgkin lymphoma that arises from the colon or rectum.  The majority are B-cell non-Hodgkin lymphomas.


Esophageal Non-Hodgkin Lymphoma

An extranodal non-Hodgkin lymphoma that arises from the esophagus with the bulk of the mass located in the esophagus.  Dysphagia may be the presenting symptom.  The vast majority of cases are diffuse large B-cell lymphomas and B-cell lymphomas of the mucosa-associated lymphoid tissue.


Childhood Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue

An extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue that occurs during childhood.


Extranodal marginal zone B-cell lymphoma (MALT type) recurrent

The reemergence of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue after a period of remission.


Intestinal Follicular Lymphoma

A follicular lymphoma arising from the intestine.  The majority of cases occur in the small intestine, particularly the duodenum.  Usually the patients have localized disease and a favorable clinical outcome.


Burkitt Lymphoma with Plasmacytoid Differentiation

Burkitt lymphoma characterized by the presence of malignant cells with eccentric basophilic cytoplasm. The nucleoli of these cells are often single and central. This morphologic variant of Burkitt lymphoma is more often seen in patients with immunodeficiency.


Angiocentric lymphoma stage III

Ann Arbor Classification: Stage III: Involvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE,S).


non-Hodgkin's lymphoma of lacrimal gland

A non-Hodgkin lymphoma that arises from the lacrimal gland. The majority of cases are of B-cell type. The most common subtypes are mucosa-associated lymphoid tissue lymphoma, follicular lymphoma, and diffuse large B-cell lymphoma.


Burkitt's lymphoma of breast

A Burkitt lymphoma that arises from the breast.  It usually affects pregnant or lactating women and presents with bilateral breast involvement and breast swelling.


B-cell small lymphocytic lymphoma refractory

B-cell small lymphocytic lymphoma that is resistant to treatment.


Ann Arbor Stage III Mature T- and NK-Cell Lymphoma


AIDS-Related Anal Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma arising from the anus and occurring in HIV-positive patients.  Homosexual HIV-positive men have an increased risk of developing anal non-Hodgkin lymphoma in comparison to the general male population.  The majority of the cases are large cell lymphomas of B-cell phenotype.


Refractory T-Cell/Histiocyte-Rich Large B-Cell Lymphoma

T-cell/histiocyte-rich large B-cell lymphoma that is resistant to treatment.


Prostate Small Lymphocytic Lymphoma

A rare small lymphocytic lymphoma that arises from the prostate gland.


Recurrent Childhood Burkitt Lymphoma

The reemergence of Burkitt lymphoma in childhood after a period of remission.


Nasal Cavity Extranodal NK/T-Cell Lymphoma

An extranodal NK/T-cell lymphoma that arises from the nasal cavity.


Recurrent Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type

The reemergence of primary cutaneous diffuse large B-cell lymphoma, leg type after a period of remission.


Borrelia Burgdoferi-Associated Cutaneous Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue

Primary cutaneous marginal zone lymphoma of mucosa-associated lymphoid tissue that arises from chronically stimulated lymphoid tissue in the skin in response to Borrelia burgdoferi infection.


Pyothorax-Associated Lymphoma

An aggressive diffuse large B-cell lymphoma arising in the pleural cavity of patients with a history of long standing pyothorax.  Patients present with chest and back pain, fever, productive cough, dyspnea, and hemoptysis.


Ann Arbor Stage I Grade 1 Follicular Lymphoma

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Recurrent Aggressive Non-Hodgkin Lymphoma

An aggressive non-Hodgkin lymphoma that has recurred after a period of remission.


non-Hodgkin's lymphoma of nasal cavity

A primary non-Hodgkin lymphoma that affects the nasal cavity and the bulk of the tumor is in this anatomic area.  The majority of the cases are nasal type extranodal NK/T-cell lymphomas and diffuse large B-cell lymphomas.


Adult T-cell lymphoma/leukemia stage IV

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


diffuse large B-cell lymphoma activated B-cell type

A diffuse large B-cell lymphoma that is characterized by the expression of CD44, PKCbeta1, Cyclin D2, BCL-2, and IRF4/MUM1 genes.