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lymphoma

  (lĭm-fō'mə)

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Any of various usually malignant tumors that arise in the lymph nodes or in other lymphoid tissue.

Definition

Lymphoma is the name of a diverse group of cancers of the lymphatic system, a connecting network of glands, organs and vessels whose principle cell is the lymphocyte.

Description

When lymphoma occurs, cells in the lymphatic system grow abnormally. They divide too rapidly and grow without any order or control. Too much tissue is formed and tumors begin to grow. Because there is lymph tissue in many parts of the body, the cancer cells may involve the liver, spleen, or bone marrow.

Two general types of lymphoma are commonly recognized: Hodgkin's disease or Hodgkin's lymphoma (HD), and Non-Hodgkin's lymphoma (NHL). The two are distinguished by cell type. These differ significantly in respect of their natural histories and their response to therapy. Hodgkin's disease tends to be primarily of nodal origin. Non-Hodgkin's lymphomas, unlike HD, can spread beyond the lymphatic system.

—Kate Kretschmann

Any of a group of malignant neoplasms derived from cells endogenous to lymphoid tissue. Lymphomas are grouped into two major categories: Hodgkin's disease and non-Hodgkin's lymphomas. Lymphomas usually originate in the lymph nodes located throughout the body, but they can arise from lymphoid tissue that does not form distinct nodes, such as that in the gastrointestinal tract or lung. Determination of the specific variety of Hodgkin's disease or non-Hodgkin's lymphoma was formerly based on the appearance of the cells when examined under a light microscope. Identification now relies on the nature of the cells with respect to certain substances (antigens) that they have on their surface or within their cytoplasm. For clinical purposes, lymphomas are categorized into three grades, low, intermediate, and high, with low-grade lymphomas having the best prognosis. See also Hodgkin's disease.

The etiology of most lymphomas is unknown. In experimental and domestic animals, viruses can cause lymphomas. Burkitt's lymphoma, a type of lymphoma that is rare in the United States but relatively common in children of central Africa, is thought to be caused by Epstein-Barr virus, a member of the herpes virus group. A form of T-cell lymphoma that has been identified in southern Japan has been attributed to a retrovirus referred to as human T-cell lymphoma-leukemia virus type 1 (HTLV-1). See also Epstein-Barr virus; Retrovirus.

Patients with lymphomas may have painless swelling of various lymph nodes, such as those in the neck or near the armpit. Some patients, especially those with Hodgkin's disease, are referred to as B symptoms (fever, malaise, and weight loss). If the lymphoma originates in lymphoid tissue outside the lymph nodes, abdominal pain will signal lymphoma of the gastrointestinal tract and a cough will point to lymphoma of the lung.

Lymph nodes involved by lymphoma are characteristically enlarged. They may be firm and have a consistency resembling fish flesh. In rare cases they are rock hard and they may show areas of cellular death (necrosis).

By using modern immunologic techniques, most lymphomas can be identified as B-cell, T-cell, or M-cell type; about 90% of lymphomas are of B-lymphocyte origin. A T-cell lymphoma that occurs in the skin, referred to as mycosis fungoides, is a lymphoma of a specific subtype of lymphocyte labeled a T-helper/inducer lymphocyte. It is that subtype that is depleted in patients with acquired immune deficiency syndrome (AIDS). See also Acquired immune deficiency syndrome (AIDS); Cellular immunology; Immunology.

Most diagnoses of lymphoma are made by surgical removal of a lymph node. Once a diagnosis of lymphoma is established, the patient usually must undergo a series of staging studies. These include a liver-spleen scan to determine if those organs are involved as well as a bone marrow biopsy to check for the presence of malignant cells. See also Oncology.

The treatment of lymphomas depends on the type of lymphoma diagnosed. Lymphomas in the low-grade group are usually not treated, since treatment does not increase life expectancy. Patients who have Hodgkin's disease or who have intermediate or high-grade non-Hodgkin's lymphoma are usually treated with chemotherapy with or without concurrent radiation. Most cases of Hodgkin's disease and more than half the cases of intermediate and high-grade non-Hodgkin's lymphomas are potentially curable. Persons with low-grade non-Hodgkin's lymphomas usually have a life expectancy of 7–10 years, although some live considerably longer. Treatment protocols are now being developed for the low-grade lymphomas in the hope of increasing life expectancy. See also Chemotherapy; Lymphatic system.

Any neoplasm made up of lymphoid tissue.

For more information on lymphoma, visit Britannica.com.

Pertaining to, or of the nature of, lymphoma.

Lymphoma
Classification & external resources

ICD-10	C81.-C96.
ICD-O:	9590-9999
MeSH	D008223

This article is about lymphoma in humans. For the disease in dogs, cats, and ferrets, see lymphoma in animals.

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

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.

• • 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])

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

1. ^ ^{a b} Pathology and Genetics of Haemo (World Health Organization Classification of Tumours S.). Oxford Univ Pr. ISBN 92-832-2411-6. 
2. ^ 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. 

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. 

Pathology: hematology (primarily C81-C96/200-208, D45-D47, D50-D77/280-289)
WBCs	hematological malignancy (Lymphoma, leukemia) -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: Warm autoimmune hemolytic anemia, HUS, MAHA, PNH aplastic anemia: Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia • Sideroblastic anemia • Hemochromatosis
Coagulation/platelets	coagulopathy: DIC • Hemophilia (A, B, C, XIII) • Von Willebrand disease Purpura: Henoch-Schönlein, ITP, TTP primary hypercoagulable state: Protein C deficiency - Protein S deficiency - Antithrombin III deficiency 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

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

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