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Lymphoma

**Lymphoma**
*Classification & external resources*
ICD-10	C81.-C96.
ICD-O:	9590-9999
MeSH	D008223

Lymphoma is a type of cancer that originates in lymphocytes (a type of white blood cell in the vertebrate immune system). There are many types of lymphoma. Lymphomas are part of the broad group of diseases called hematological neoplasms.

In the 19th and 20th centuries the affliction was called Hodgkin's Disease, as it was discovered by Thomas Hodgkin in 1832. Colloquially, lymphoma is broadly categorized as Hodgkin's lymphoma and non-Hodgkin lymphoma (all other types of lymphoma). Scientific classification of the types of lymphoma is more detailed.

Although older classifications referred to histiocytic lymphomas, these are recognized in newer classifications as of B, T or NK cell lineage. Histiocytic malignancies are rare and are classified as sarcomas.^[1]

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Prevalence

According to the U.S. National Institutes of Health, lymphomas account for about five percent of all cases of cancer in the United States, and Hodgkin's lymphoma in particular accounts for less than one percent of all cases of cancer in the United States.

Because the lymphatic system is part of the body's immune system, patients with a weakened immune system, such as from HIV infection or from certain drugs or medication, also have a higher incidence of lymphoma.

Classification

WHO classification

The WHO Classification is the latest classification of lymphoma, published by the World Health Organization in 2001.^[1] It was based upon the "Revised European-American Lymphoma classification" (REAL).

This classification attempts to classify lymphomas by cell type, i.e. the normal cell type that most closely resembles the tumor. They are classified in three large groups: the B cell tumors, the T cell and natural killer cell tumors, Hodgkin lymphoma, and other less common groups: (ICD-O codes are provided where available).

Mature B cell neoplasms

- Chronic lymphocytic leukemia/Small lymphocytic lymphoma
- B-cell prolymphocytic leukemia
- Lymphoplasmacytic lymphoma/Waldenström macroglobulinemia
- Splenic marginal zone lymphoma
- Plasma cell neoplasms
  - Plasma cell myeloma
  - Plasmacytoma
  - Monoclonal immunoglobulin deposition diseases
  - Heavy chain diseases
- Extranodal marginal zone B cell lymphoma (MALT lymphoma)
- Nodal marginal zone B cell lymphoma
- Follicular lymphoma
- Mantle cell lymphoma
- Diffuse large B cell lymphoma
- Mediastinal (thymic) large B cell lymphoma
- Intravascular large B cell lymphoma
- Primary effusion lymphoma
- Burkitt lymphoma/leukemia
- Lymphomatoid granulomatosis

Mature T cell and natural killer (NK) cell neoplasms

- T cell prolymphocytic leukemia
- T cell large granular lymphocytic leukemia
- Aggressive NK cell leukemia
- Adult T cell leukemia/lymphoma
- Extranodal NK/T cell lymphoma, nasal type
- Enteropathy-type T cell lymphoma
- Hepatosplenic T cell lymphoma
- Blastic NK cell lymphoma
- Mycosis fungoides / Sezary syndrome
- Primary cutaneous CD30-positive T cell lymphoproliferative disorders
  - Primary cutaneous anaplastic large cell lymphoma
  - Lymphomatoid papulosis
- Angioimmunoblastic T cell lymphoma
- Peripheral T cell lymphoma, unspecified
- Anaplastic large cell lymphoma

Hodgkin lymphoma

- Nodular lymphocyte-predominant Hodgkin lymphoma
- Classical Hodgkin lymphoma
  - Nodular sclerosis
  - Mixed cellularity
  - Lymphocyte-rich
  - Lymphocyte depleted or not depleted

Immunodeficiency-associated lymphoproliferative disorders

- Associated with a primary immune disorder
- Associated with the Human Immunodeficiency Virus (HIV)
- Post-transplant
- Associated with Methotrexate therapy

Working formulation

The Working Formulation, published in 1982, is primarily descriptive. It is still occasionally used, but has been superseded by the WHO classification, above.

Low grade

Malignant Lymphoma, small lymphocytic (chronic lymphocytic leukemia)
Malignant Lymphoma, follicular, predominantly small cleaved cell
Malignant Lymphoma, follicular, mixed (small cleaved and large cell)

High grade

Malignant Lymphoma, large cell, immunoblastic
Malignant Lymphoma, lymphoblastic
Malignant Lymphoma, small non-cleaved cells (Burkitt's lymphoma)

Miscellaneous

Composite
Mycosis fungoides
Histiocytic
Extramedullary plasmacytoma
Unclassifiable

Other classification systems

ICD-O (codes 9590-9999, details at [1]) (archive link, was dead)
ICD-10 (codes C81-C96, details at [2])

Diagnosis, etiology, staging, prognosis, and treatment

See separate links to Hodgkin's lymphoma and non-Hodgkin's lymphoma.

Genetics

Enteropathy associated T-cell lymphoma (EATL) is environmentally induced as a result of the consumption of Triticeae glutens. In gluten sensitive individuals with EATL 68% are homozygotes of the DQB1*02 subtype at the HLA-DQB1 locus (serotype DQ2).^[2] (See Coeliac Disease, HLA-DQ, HLA DR3-DQ2)

References

^ ^a ^b Pathology and Genetics of Haemo (World Health Organization Classification of Tumours S.). Oxford Univ Pr. ISBN 92-832-2411-6.
^ Al-Toma A, Verbeek WH, Hadithi M, von Blomberg BM, Mulder CJ (2007). "Survival in Refractory Coeliac Disease and Enteropathy associated T cell Lymphoma: Retrospective evaluation of single centre experience". doi:10.1136/gut.2006.114512. PMID 17470479.

Research Foundations

Lymphoma Research Foundation

Information

MedlinePlus: Lymphoma
ICD10 classification of lymphoma
About Lymphoma: A guide to lymphoma for patients
"Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues"
Lymphomas in Children and Adolescents The Centre for Cancer and Blood Disorders at Sydney Children’s Hospital provides information on cancers in children and adolescents, including Hodgkins Disease and Non-Hodgkins lymphomas.
Summary at NIH/National Cancer Institute
Non-Hodgkin's Lymphoma Symptoms & Support Information

Societies and Support Groups

The Leukemia & Lymphoma Society
A Lot of Life! Positive Stories from a Survivor at Large
Lymphoma Support Groups
Lymphoma Support Ireland - Booklets & Patient Stories online
Support Group web site for the Coventry Lymphoma Association Support Group in the UK

Statistics

US Lymphoma Fact Sheet from the American Cancer Society
UK Hodgkin Lymphoma Statistics
UK Non-Hodgkin Lymphoma Statistics
About Lymphomas - Patients Against Lymphoma
Timeline of discovery and treatment of Hodgkin's Lymphoma
National Cancer Institute. "Risk of Lymphoma Increases with Hepatitis C Virus Infection", May 2007.

v • d • e Pathology: hematology (primarily C81-C96/200-208, D45-D47, D50-D77/280-289)
WBCs	hematological malignancy (lymphoma, leukemia, multiple myeloma), myeloproliferative disease, myelodysplastic syndrome -cytosis (Agranulocytosis, Leukocytosis, Lymphocytosis, Monocytosis) • -penia (Lymphopenia, Neutropenia)
RBCs/anemia/ hemoglobinopathy	nutritional anemia: Iron deficiency anemia, Plummer-Vinson syndrome, Megaloblastic anemia (Pernicious anemia) hereditary hemolytic anemia: G6PD Deficiency, Thalassemia, Sickle-cell disease/trait, Hereditary spherocytosis, Hereditary elliptocytosis, Hereditary stomatocytosis acquired hemolytic anemia: Autoimmune (Warm), HUS, MAHA, PNH, PCH aplastic anemia: Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia • Sideroblastic anemia • Hemochromatosis
Coagulation/platelets	coagulopathy: DIC • Hemophilia (A/VII, B/IX, C/XI, XIII) • Von Willebrand disease Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP primary hypercoagulable state: Protein C deficiency - Protein S deficiency - Antithrombin III deficiency - Antiphospholipid syndrome - Factor V Leiden other hemorrhagic conditions: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Grey platelet syndrome
Histiocytosis	WHO-I Langerhans cell histiocytosis - non-Langerhans-cell histiocytosis/WHO-II (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) - malignant histiocytic disorders/WHO-III (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease)
Other	Asplenia/hyposplenism - Methemoglobinemia

v • d • e Hematological malignancy histology (ICD-O 9590-9989)
Lymphomas (9590-9759)	Hodgkin's lymphoma vs. Non-Hodgkin lymphoma - Diffuse lymphoma vs. Follicular lymphoma B-cell lymphoma (Small cell, Primary effusion, Diffuse large, ,Burkitt's, Splenic marginal zone, MALT) T-cell lymphoma (Cutaneous , Mycosis fungoides/Sézary's disease, Angioimmunoblastic, Anaplastic large cell, Hepatosplenic) plasma cell (Plasmacytoma, Multiple myeloma) mast cell tumor (Mast-cell sarcoma, Malignant mastocytosis, Malignant histiocytosis, Langerhans cell histiocytosis)
Immunoproliferative disorders (9760-9799)	Waldenström macroglobulinemia - Lymphomatoid granulomatosis
Lymphoid leukemias (9800-9839)	ALL - CLL - T-cell leukemia (Adult, Large granular lymphocyte, Prolymphocytic, Acute lymphoblastic) - B-cell leukemia (Prolymphocytic)
Myeloid leukemias (9840-9939, 9963)	AML (M2, APL/M3, AMoL/M5, Erythroleukemia/M6) - CML (CMoL, CNL, Philadelphia chromosome) - Granulocytic sarcoma
Other leukemias (9940-9949)	Hairy cell leukemia - Aggressive NK-cell leukemia
Myeloproliferative disease (9950-9961)	Polycythemia vera - Essential thrombocytosis - Myelofibrosis
Other (9964-9989)	Hypereosinophilic syndrome - Post-transplant lymphoproliferative disorder - Myelodysplastic syndrome

Categories: Blood disorders | Types of cancer

This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Lymphoma". A list of authors is available in Wikipedia.