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Lymphoma is a cancer that begins in the lymphocytes of the immune system and presents as a solid tumor of lymphoid cells. They often originate like balls in lymph nodes, presenting as an enlargement of the node (a tumor). Lymphomas are closely related to lymphoid leukemias, which also originate in lymphocytes but typically involve only circulating blood and the bone marrow (where blood cells are generated in a process termed haematopoesis) and do not usually form static tumours. There are many types of lymphomas, and in turn, lymphomas are a part of the broad group of diseases called hematological neoplasms.

Thomas Hodgkin published in 1832 the first description of lymphoma, specifically of the form named after him, Hodgkin's lymphoma. Since then many other forms of lymphoma have been described, grouped under several proposed classifications. The 1982 Working formulation classification became very popular. It introduced the category non-Hodgkin lymphoma (NHL), itself divided into 16 different diseases. However, since these different lymphomas have little in common with each other, the NHL label is of limited usefulness for doctors or patients and is slowly being abandoned. The latest classification by the WHO (2001) lists 43 different forms of lymphoma divided in four broad groups.

Some forms of lymphoma are categorized as indolent (e.g. small lymphocytic lymphoma), compatible with a long life even without treatment, whereas other forms are aggressive (e.g. Burkitt's lymphoma), causing rapid deterioration and death. However, most of the aggressive lymphomas respond well to treatment and are curable. The prognosis therefore depends on the correct classification of the disease, established by a pathologist after examination of a biopsy.

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

Classification

Kiel classification

As an alternative to the American Lukes-Butler classification, in the early 1970s, Karl Lennert of Kielmarker, Germany, proposed a new system of classifying lymphomas based on cellular morphology and their relationship to cells of the normal peripheral lymphoid system.

REAL classification

In the mid 1990s, the Revised European-American Lymphoma (REAL) Classification attempted to apply immunophenotypic and genetic features in identifying distinct clinicopathologic NHL entities.

WHO classification

The WHO Classification, published in 2001 and updated in 2008, is the latest classification of lymphoma and is based upon the foundations laid within the "Revised European-American Lymphoma classification" (REAL). This system attempts to group lymphomas by cell type (i.e. the normal cell type that most resembles the tumour) and defining phenotypic, molecular or cytogenetic characteristics. There are three large groups: the B cell, T cell, and natural killer cell tumours. Other less common groups, are also recognized. Hodgkin's lymphoma, although considered separately within the WHO (and preceding) classifications, is now recognized as being a tumour of, albeit markedly abnormal, lymphocytes of mature B cell lineage.

Mature B cell neoplasms

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 underexpressed in lymphoma cells (as compared to normal cells), whereas red indicates genes that are overexpressed in lymphoma cells.




Mature T cell and natural killer (NK) cell neoplasms



Hodgkin lymphoma



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 and Non-Hodgkin lymphoma

The 1982 Working Formulation is a classification of Non-Hodgkin Lymphoma. It has since been replaced by other lymphoma classifications, the latest published by the WHO in 2001 (updated in September 2008), but is still used by cancer agencies for compilation of lymphoma statistics.

Other classification systems



Cause

Association of medications with lymphoma

Inflammatory Bowel Disease (IBD) patients treated with the cancer medications, 6-Mercaptopurine (Purinethol) and Azathioprine (Imuran) are at a greater risk for developing lymphoma. This risk is 4 times higher than the general population.

Diagnosis, etiology, staging, prognosis, and treatment

See the article of the corresponding form of lymphoma.

Epidemiology

[[Image:Lymphomas, multiple myeloma world map - Death - WHO2004.svg|thumb|Age-standardized death from lymphomas and multiple myeloma per 100,000 inhabitants in 2004.


]]Lymphoma is the most common form of hematological malignancy, or "blood cancer", in the developed world.

Taken together, lymphomas represent 5.3% of all cancers (excluding simple basal cell and squamous cell skin cancers) in the United States, and 55.6% of all blood cancers.

According to the U.S.marker National Institutes of Healthmarker, 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 whole 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.

See also



References

  1. Wagman LD. "Principles of Surgical Oncology" in Pazdur R, Wagman LD, Camphausen KA, Hoskins WJ (Eds) Cancer Management: A Multidisciplinary Approach. 11 ed. 2008.
  2. www.emedicine.com on Lymphoma, Non-Hodgkin


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