Waldenstrom Macroglobulinemia
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. Asymptomatic patients may be observed initially, whereas durable responses have been observed in those requiring chemotherapy. The Bruton tyrosine kinase inhibitor ibrutinib is also approved for treatment of WM with an overall response rate of 62%.
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