Lymphoma

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Lymphoma Classification and external resources
Lymph node: Malignant Lymphoma Large Cell Type: Gross natural color excellent view of cut mesentery showing massively enlarged mesenteric nodes with focal hemorrhages case diagnosed several years ago as reticulum cell sarcoma excellent demonstration of nodes with lymphoma. Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology
ICD-10	C81.-C96.
ICD-O:	9590-9999
MeSH	D008223

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Overview

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]

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 minor 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

DNA-microarray analysis of Burkitt's lymphoma and diffuse large B-cell lymphoma (DLBCL) showing differences in gene expression patterns. Colors indicate levels of expression; green indicates genes that are overexpressed in normal cells compared to lymphoma cells and red indicates genes that are overexpressed in lymphoma cells compared to normal cells.

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 [3])
• ICD-10 (codes C81-C96, details at [4])

For diagnosis, etiology, staging, prognosis, and treatment

Please 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)

See also

• Hodgkin's lymphoma
• Non-Hodgkin's lymphoma
• Follicular lymphoma
• Burkitt's lymphoma
• Mantle cell lymphoma
• Gastric lymphoma
• Cutaneous T Cell lymphoma
• Mycosis fungoides
• Anaplastic large cell lymphoma
• MALT lymphoma
• Primary central nervous system lymphoma
• BCP-1 cells
• Ann Arbor staging
• International Prognostic Index

References

External links

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. 

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