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Lymphoma - non-Hodgkin's; NHL; B-cell lymphomas
Lymphomas are malignancies of the lymph system that are generally subdivided into two groups, Hodgkin's disease (HD) and non-Hodgkin's lymphoma (NHL). Hodgkin's disease accounts for about 15% of all lymphomas. [For more information, see In-Depth Report #83: Hodgkin's disease.]
Non-Hodgkin's lymphomas is a term for malignancies that range from a very slow disease to an extremely aggressive but curable condition. They have certain features in common.
Lymphomas, such as non-Hodgkin's lymphomas and Hodgkin's disease, represent tumors of the lymphatic system. This system is a network of organs, ducts, and nodes. The system interacts with the blood's circulatory system to transport a watery clear fluid called lymph throughout the body. The lymphatic system contains lymphocytes, important cells involved in defending the body against infectious organisms. This system also restores 60% of the fluid that leaks out from blood capillaries back into circulation, and its ducts provide transportation for fats, proteins, and other substances collected from the body's tissues.
Lymphocytes. The lymphatic system is involved in the production and transportation of lymphocytes, white blood cells that are a primary component of the immune system. Among other vital functions, certain lymphocytes are responsible for producing antibodies, factors that can target and attack specific foreign proteins (antigens).
Lymph Nodes. In the lymph node, lymphocytes receive their initial exposure to foreign substances (antigens), such as bacteria or other microorganisms, activating the lymphocytes to perform their immune functions. The size of a lymph node varies generally from that of a pinhead to a bean. Most nodes are in clusters located throughout the system. Important node clusters are found in the neck, lower arm, armpit, and groin.
Other Structures in the Lymphatic System. The tonsils and adenoids are secondary organs composed of masses of lymph tissue that also play a role in the lymphatic system. The spleen is another important organ that processes lymphocytes from incoming blood.
Non-Hodgkin's lymphomas occur most often in lymph nodes in the chest, neck, abdomen, tonsils, and the skin. NHLs may also develop in sites other than lymph nodes such as the digestive tract, central nervous system, and around the tonsils.
There are more than 30 distinct types of non-Hodgkin's lymphomas. Lymphomas are categorized in a several ways:
Common types of B-cell lymphoma include:
Diffuse Large B-Cell Lymphoma (DLBLC). DLBCL is the most common type of non-Hodgkinâ ' s lymphoma, accounting for about 30% of all NHL cases. It is an aggressive, fast-growing lymphoma that usually affects adults but can also occur in children. DLBCL can occur in lymph nodes or in organs outside of the lymphatic system. DLBCL includes several subtypes such as mediastinal large B-cell lymphoma, intravascular large B-cell lymphoma, and primary effusion lymphoma.
Follicular Lymphoma (FLs). Follicular lymphoma is the second most common type lymphoma, accounting for about 20% of all NHL cases. It is usually indolent (slow growing) but about half of follicular lymphomas transform over time into the aggressive diffuse large B-cell lymphoma.
Mantle Cell Lymphoma. Mantle cell lymphoma is an aggressive type of lymphoma that represent about 7% of NHL cases. It is a difficult type of lymphoma to treat and often does not respond to chemotherapy. It is found in lymph nodes, the spleen, bone marrow, and gastrointestinal system. Mantle cell lymphoma usually develops in men over age 60.
Small Lymphocytic Lymphoma (SLL). SLL is an indolent type of lymphoma that is closely related to B-cell chronic lymphocytic leukemia (CLL). It accounts for about 5% of NHL cases.
Marginal Zone Lymphomas (MZL). MZLs are categorized depending on where the lymphoma is located. Mucosa-associated lymphoid tissue lymphomas (MALT) usually involve the gastrointestinal tract, thyroid, lungs, saliva glands, or skin. MALT is often associated with a history of an autoimmune disorder (such as Sjogren syndrome in the salivary glands or Hashimoto's thyroiditis in the thyroid gland). MALT is also associated with bacterial infection in the stomach (H. pylori ) and can be potentially cured by antibiotics when treated in its early stages. Splenic marginal zone lymphoma affects the spleen, blood, and bone marrow. Nodal marginal zone B-cell lymphoma is a rare type of indolent lymphoma that involves the lymph nodes.
Lymphoplasmacytic Lymphoma. Lymphoplasmacytic lymphoma, also called Waldenstrom's macroglobulinemia or immunocytoma, is a rare type of lymphoma accounting for about 1% of NHL cases. It usually affects older adults and most often involves bone marrow, lymph nodes, and spleen.
Primary Central Nervous System Lymphoma. This lymphoma involves the brain and spinal cord. Although it is generally rare, it is common in people who have AIDS.
Burkitt's Lymphoma. This is one of the most common types of childhood NHL, accounting for about 40% of NHL pediatric cases in the United States. It usually starts in the abdomen and spreads to other organs, including the brain. In African children, it often involves facial bones and is associated with Epstein-Barr infection.
Lymphoblastic Lymphoma. This lymphoma is also common in children, accounting for about 25% of NHL pediatric cases, most often boys. It is associated with a large mediastinal mass (occurring in chest cavity between the lungs) and carries a high risk for spreading to bone marrow, the brain, and other lymph nodes.
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