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lym·pho·ma (lĭm-fō'mə) ![]() |
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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
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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.
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Pertaining to, or of the nature of, lymphoma.
| Wikipedia: Lymphoma |
| Lymphoma | |
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| Classification and external resources | |
Gastric MALT lymphoma |
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| ICD-10 | C81.-C96. |
| ICD-O: | 9590-9999 |
| MeSH | D008223 |
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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.[1] 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.[1] 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.[2] 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.[3]
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.[4]
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As an alternative to the American Lukes-Butler classification, in the early 1970s, Karl Lennert of Kiel, Germany, proposed a new system of classifying lymphomas based on cellular morphology and their relationship to cells of the normal peripheral lymphoid system.[5]
In the mid 1990s, the Revised European-American Lymphoma (REAL) Classification attempted to apply immunophenotypic and genetic features in identifying distinct clinicopathologic NHL entities.[6]
The WHO Classification, published in 2001 and updated in 2008,[4] 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.
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.
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. [7]
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See the article of the corresponding form of lymphoma.
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.[9]
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 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.
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