Journal
EXPERT REVIEW OF HEMATOLOGY
Volume 14, Issue 7, Pages 621-632Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1080/17474086.2021.1947236
Keywords
Bing-Neel syndrome; bendamustine; bortezomib; hyperviscosity syndrome; ibrutinib; monoclonal IgM; peripheral neuropathy; rituximab; venetoclax; Waldenstrom's macroglobulinemia
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The availability of many novel, active and less toxic regimens for the treatment of WM allows the management of the disease with strategies that depend on clinical presentation and disease-related complications, age, toxicity considerations, and presence of comorbidities.
Introduction: Waldenstrom's macroglobulinemia (WM) is a rare lymphoma characterized by the accumulation of IgM-secreting lymphoplasmacytic cells in the bone marrow and other organs. Clinical sequelae relate to direct tissue infiltration by malignant cells but also to the physicochemical and immunological properties of the monoclonal IgM, resulting in a variety of disease-related complications. Areas covered: This narrative review, following a thorough Pubmed search of pertinent published literature, discusses complications secondary to WM, related to direct tumor infiltration, monoclonal IgM circulation, and deposition, as well as other less common ones. The description and pathophysiology of these complications were described together with their specific management strategies and in the context of available treatment options for WM (anti-CD20 monoclonal antibody-based combinations, proteasome inhibitors, BTK inhibitors, and other emerging ones). Expert opinion: The availability of many novel, active and less toxic regimens for the treatment of WM allows the management of the disease with strategies that depend on clinical presentation and disease-related complications, age, toxicity considerations, and presence of comorbidities.
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