Journal
CLINICAL LYMPHOMA MYELOMA & LEUKEMIA
Volume 17, Issue 5, Pages 252-262Publisher
CIG MEDIA GROUP, LP
DOI: 10.1016/j.clml.2017.02.028
Keywords
CXCR4; Lymphoid neoplasm; Lymphoplasmacytic lymphoma; Monoclonal immunoglobulin M; MYD88
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Funding
- National Cancer Institute, National Institutes of Health, Bethesda [P30 CA023074, T35 HL07479]
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Waldenstrom macroglobulinemia (WM) is a low-grade B-cell clonal disorder characterized by lymphoplasmacytic bone marrow involvement associated with monoclonal immunoglobulin M. Although WM remains to be an incurable disease with a heterogeneous clinical course, the recent discovery of mutations in the MYD88 and CXCR4 genes further enhanced our understanding of its pathogenesis. Development of new therapies including monoclonal antibodies, proteasome inhibitors, and Bruton tyrosine kinase inhibitors have made the management of WM increasingly complex. Treatment should be tailored to the individual patient while considering many clinical factors. The clinical outcomes are expected to continue to improve, given the emergence of novel therapeutics and better understanding of the underlying pathogenesis. (C) 2017 Elsevier Inc. All rights reserved.
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