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Non-Hodgkin's Lymphoma

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Cellular Classification


The management of lymphoplasmacytic lymphoma is similar to that of other low-grade lymphomas, especially diffuse small lymphocytic lymphoma/chronic lymphocytic leukemia.[26,27,28,29,30] If the viscosity relative to water is greater than four, the patient may have manifestations of hyperviscosity. Plasmapheresis is useful for temporary, acute symptoms (such as retinopathy, congestive heart failure, and CNS dysfunction) but should be combined with chemotherapy for prolonged control of the disease. Symptomatic patients with a serum viscosity of not more than four are usually started directly on chemotherapy. Therapy may be required to correct hemolytic anemia in patients with chronic cold agglutinin disease; chlorambucil, with or without prednisone, is the mainstay. Occasionally, a heated room is required for patients whose cold agglutinins become activated by even minor chilling.

Asymptomatic patients can be monitored for evidence of disease progression without immediate need for chemotherapy.[18] First-line regimens include rituximab, the nucleoside analogs, and alkylating agents, either as single agents or as part of combination chemotherapy.[31] Rituximab shows 60% to 80% response rates in previously untreated patients, but close monitoring of the serum IgM is required because of a sudden rise in this paraprotein at the start of therapy.[31,32,33][Level of evidence: 3iiiDiii] The nucleoside analogues 2-chlorodeoxyadenosine and fludarabine have shown similar response rates for previously untreated patients with lymphoplasmacytic lymphoma.[34,35,36][Level of evidence: 3iiiDiii] Single-agent alkylators and combination chemotherapy also show similar response rates.[37][Level of evidence: 3iiiDiii] Currently, no randomized trials guide clinicians about the initial choice of rituximab, nucleoside analogs, alkylating agents, combination chemotherapy, or combinations of these options.[27,31]

Interferon-a also shows activity in this disease, in contrast to poor responses in patients with multiple myeloma.[38] Myeloablative therapy with autologous hematopoietic stem cell support is under clinical evaluation.[39,40] Candidates for this approach should avoid long-term use of alkylating agents or purine nucleoside analogs, which can deplete hematopoietic stem cells.[31] After relapse from alkylating-agent therapy, 92 patients with lymphoplasmacytic lymphoma were randomized to fludarabine versus cyclophosphamide, doxorubicin, and prednisone. Although relapse-free survival favored fludarabine (median duration 19 months vs. 3 months, P < .01), no difference was observed in OS.[41][Level of evidence: 1iiDi] Among patients with concomitant hepatitis C virus (HCV) infection, some will attain a complete or partial remission after loss of detectable HCV RNA with treatment using interferon-a with or without ribavirin.[42][Level of evidence: 3iiiDiii]


Marginal zone lymphomas were previously included among the diffuse small lymphocytic lymphomas. When marginal zone lymphomas involve the nodes, they are called monocytoid B-cell lymphomas or nodal marginal zone B-cell lymphomas, and when they involve extranodal sites (e.g., gastrointestinal tract, thyroid, lung, breast, orbit, and skin), they are called MALT lymphomas.[4,43,44,45,46,47,48,49,50,51]


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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