Waldenström Macroglobulinemia

Steven P. Treon, Zachary R. Hunter, Jorge J. Castillo, Giampaolo Merlini

Research output: Contribution to journalArticle

Abstract

Waldenström macroglobulinemia (WM) is an IgM-secreting B-cell lymphoproliferative disorder, with strong familial predisposition. MYD88 L265P and CXCR4 WHIM mutations are common in WM and support the growth and survival of WM cells. Clinical manifestations of disease are related to both tumor cell infiltration and paraprotein production. Current treatment includes monoclonal antibodies, alkylating agents, nucleoside analogs, proteasome inhibitors, immunomodulatory drugs, and signal inhibitors. Short- and long-term toxicities should be weighed in treatment decisions with use of these agents. Elucidation of the signaling pathways involved in WM is helping to advance targeted therapeutics for WM and includes efforts directed at MYD88 and CXCR4 signaling.

Original languageEnglish
Pages (from-to)945-970
Number of pages26
JournalHematology/Oncology Clinics of North America
Volume28
Issue number5
DOIs
Publication statusPublished - Oct 1 2014

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Keywords

  • CXCR4 WHIM mutations
  • Lymphoproliferative disorder
  • Morbidity
  • MYD88 L265P
  • Treatment options
  • Waldenström macroglobulinemia

ASJC Scopus subject areas

  • Oncology
  • Hematology
  • Medicine(all)

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