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Epidemiology, pathogenesis, clinical manifestations, and diagnosis 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. This broad definition includes patients with monoclonal gammopathy of undetermined significance of the IgM type (IgM MGUS), smoldering Waldenström macroglobulinemia, Waldenström macroglobulinemia (WM), and a number of related disorders in which an IgM monoclonal protein is detected, such as chronic lymphocytic leukemia (CLL), a number of lymphoma variants, and primary (AL) amyloidosis.

WM is a distinct clinicopathologic entity demonstrating lymphoplasmacytic lymphoma (LPL) in the bone marrow with an IgM monoclonal gammopathy in the blood. Patients may present with symptoms related to the infiltration of the hematopoietic tissues or the effects of monoclonal IgM in the blood.

This topic review will limit discussion to the clinical manifestations and diagnosis of WM. The pathologic features of LPL and the prognosis and treatment of WM are discussed separately.

(See "Treatment and prognosis of Waldenström macroglobulinemia".)

(See "Clinical manifestations, pathologic features, and diagnosis of lymphoplasmacytic lymphoma".)

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Literature review current through: Nov 2017. | This topic last updated: Dec 01, 2017.
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