Waldenstrom macroglobulinemia

Abstract

Waldenström's macroglobulinemia (WM), also called lymphoplasmacytic lymphoma, is a rare hematologic disorder associated with a monoclonal immunoglobulin M protein (IgM) that infrequently involves the kidney. Renal manifestations are primarily glomerular abnormalities related to paraprotein deposition, but direct infiltration of the neoplastic hematopoietic cells may also be seen. Treatment is mandatory after the diagnosis of WM in symptomatic patients due primarily to complications of hyperviscosity, but no standard approach for therapy has been described. An exciting advancement in the understanding of the pathogenesis ofWMis the discovery of a somatic mutation in the myeloid differentiation primary response gene 88 (MYD88) gene resulting in activation of Bruton's tyrosine kinase and leading to survival of the neoplastic cells. This also serves as a point of therapy as a novel drug, ibrutinib, a Bruton's tyrosine kinase (BTK) inhibitor, has shown great response in the treatment of WM.