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Treatment and prognosis of Waldenström macroglobulinemia

S Vincent Rajkumar, MD
Stephen M Ansell, MD, PhD
Section Editor
Robert A Kyle, MD
Deputy Editor
Rebecca F Connor, MD


The term "macroglobulinemia" refers to the production of excess IgM monoclonal protein that occurs in certain clonal lymphoproliferative disorders and plasma cell dyscrasias. Waldenström macroglobulinemia (WM) is a distinct clinicopathologic entity demonstrating lymphoplasmacytic lymphoma (LPL) in the bone marrow with an IgM monoclonal gammopathy (macroglobulinemia) in the blood. Patients may present with symptoms related to the infiltration of the lymph nodes and spleen or the effects of monoclonal IgM in the blood.

There is no standard therapy for the treatment of symptomatic WM. Whenever possible, patients should be encouraged to participate in clinical trials. This topic review presents suggestions for the treatment of patients who are not eligible for clinical trials or who choose not to participate in clinical trials.

The treatment and prognosis of WM will be reviewed here. The clinical manifestations and diagnosis of this disorder are discussed separately. (See "Epidemiology, pathogenesis, clinical manifestations, and diagnosis of Waldenström macroglobulinemia" and "Clinical manifestations, pathologic features, and diagnosis of lymphoplasmacytic lymphoma".)


The initial evaluation of patients with WM must establish the diagnosis, the extent of disease, the presence of associated conditions, and the performance status of the patient. Particular attention should be paid in the history and physical examination to the following:

Symptoms of hyperviscosity (eg, epistaxis, bleeding gums, visual changes, headache, dizziness). Funduscopic examination should be performed in all patients with symptoms of hyperviscosity and/or IgM ≥3000 mg/dL. (See "Epidemiology, pathogenesis, clinical manifestations, and diagnosis of Waldenström macroglobulinemia", section on 'Hyperviscosity syndrome'.)

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Literature review current through: Nov 2017. | This topic last updated: Oct 30, 2017.
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