Waldenström Macroglobulinemia

Waldenström Macroglobulinemia is a topic covered in the Washington Manual of Medical Therapeutics.

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WM is an uncommon IgM monoclonal disorder also known as lymphoplasmacytic lymphoma, characterized by mild hematologic abnormalities, and accompanied by tissue infiltration including lymphadenopathy, splenomegaly, or hepatomegaly. Because of its high molecular weight and concentration, IgM gammopathy can lead to hyperviscosity (central nervous system, visual, cardiac) manifestations. In these cases, emergent plasmapheresis to decrease IgM concentration is indicated. The MYD88 L265P mutation is a commonly recurring mutation in patients with WM and can be useful in differentiating WM from B-cell disorders that have similar features.1 Asymptomatic patients may be observed initially, whereas durable responses have been observed in those requiring chemotherapy.2 The Bruton tyrosine kinase inhibitor ibrutinib received FDA approval in January 2015 for treatment of WM based on an overall response rate of 62% in a study of 63 patients with WM.3

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