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Non-Hodgkin's Lymphoma

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Cellular Classification

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INTRAVASCULAR LARGE B-CELL LYMPHOMA (INTRAVASCULAR LYMPHOMATOSIS)

Intravascular lymphomatosis is characterized by large cell lymphoma confined to the intravascular lumen; with the use of aggressive combination chemotherapy, the prognosis is similar to more conventional presentations.[161] The brain, kidneys, lungs, and skin are the organs most likely affected by intravascular lymphomatosis.

BURKITT'S LYMPHOMA/DIFFUSE SMALL NONCLEAVED-CELL LYMPHOMA

Burkitt's lymphoma/diffuse small noncleaved-cell lymphoma typically involves younger patients and represents the most common type of pediatric non-Hodgkin’s lymphoma.[162] These types of aggressive extranodal B-cell lymphomas are characterized by translocation and deregulation of the C-myc gene on chromosome 8.[163] A subgroup of patients with dual translocation of C-myc and bcl-2 appear to have an extremely poor outcome despite aggressive therapy (5-month OS).[164][Level of evidence: 3iiiA] In some patients with larger B cells, there is morphologic overlap with diffuse large B-cell lymphoma. These Burkitt's-like large cell lymphomas show C-myc deregulation, extremely high proliferation rates, and a gene-expression profile as expected for classic Burkitt's lymphoma.[10,165,166] Endemic cases, usually from Africa, involve the facial bones or jaws of children, mostly containing EBV genomes. Sporadic cases usually involve the gastrointestinal system, ovaries, or kidneys. Patients present with rapidly growing masses and a very high lactate dehydrogenase but are potentially curable with intensive doxorubicin-based combination chemotherapy. Treatment of Burkitt's lymphoma/diffuse small noncleaved-cell lymphoma involves aggressive multidrug regimens similar to those used for the advanced-stage aggressive lymphomas (diffuse large cell).[167,168,169] Aggressive combination chemotherapy, which is patterned after that used in childhood Burkitt's lymphoma, has been described and has been very successful for adult patients with more than 60% of advanced-stage patients free of disease at 5 years.[170,171,172] [173,174,175] Adverse prognostic factors include bulky abdominal disease and high serum LDH. In some institutions, treatment includes the use of consolidative bone marrow transplantation (BMT).[176,177] Patients with Burkitt's lymphoma have a 20% to 30% lifetime risk of CNS involvement. Prophylaxis with intrathecal chemotherapy is required as part of induction therapy.[178] (Refer to the PDQ summaries on Primary Central Nervous System Lymphoma Treatment and AIDS-Related Lymphoma Treatment for more information.)

LYMPHOBLASTIC LYMPHOMA

Lymphoblastic lymphoma (precursor T-cell) is a very aggressive form of NHL. It often occurs in young patients but not exclusively.[179] It is commonly associated with large mediastinal masses and has a high predilection for disseminating to bone marrow and to the CNS. Treatment is usually patterned after that for acute lymphoblastic leukemia. Intensive combination chemotherapy with or without BMT is the standard treatment of this aggressive histologic type of NHL.[180,181,182] Radiation therapy is sometimes given to areas of bulky tumor masses. Since these forms of NHL tend to progress so quickly, combination chemotherapy is instituted rapidly once the diagnosis has been confirmed. Careful review of the pathologic specimens, bone marrow aspirate, biopsy specimen, cerebrospinal fluid cytology, and lymphocyte marker constitute the most important aspects of the pretreatment staging workup. (Refer to the PDQ summary on Adult Acute Lymphoblastic Leukemia Treatment for more information.)

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WebMD Public Information from the National Cancer Institute

Last Updated: April 02, 2007
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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