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Current approach to Waldenström macroglobulinemia.

Prashant Kapoor1, S Vincent Rajkumar1

  • 1Mayo Clinic, Rochester, MN, USA.

Blood Reviews
|September 2, 2023
PubMed
Summary

Waldenström macroglobulinemia (WM) management is evolving beyond chemoimmunotherapy. Bruton's tyrosine kinase (BTK) inhibitors and other targeted therapies offer new treatment paradigms for this B-cell lymphoma.

Keywords:
BTK inhibitorCXCR4ChemoimmunotherapyIgM monoclonal gammopathyLymphoplasmacytic lymphomaMYD88

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Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Waldenström macroglobulinemia (WM) is a CD20+, B-cell non-Hodgkin lymphoma with variable clinical presentations.
  • Current treatment often involves chemoimmunotherapy with rituximab, but newer agents are emerging.
  • Understanding WM pathophysiology, including MYD88 and CXCR4 mutations, is crucial for personalized treatment.

Purpose of the Study:

  • To review the evolving treatment landscape of Waldenström macroglobulinemia.
  • To highlight the integration of Bruton's tyrosine kinase (BTK) inhibitors and other novel agents.
  • To discuss the implications of mutation status on treatment decisions.

Main Methods:

  • Review of current literature on WM treatment strategies.
  • Analysis of clinical trial data for novel therapies.
  • Discussion of emerging treatment paradigms including BTK inhibitors and BCL2 inhibitors.

Main Results:

  • Rituximab is a standard addition to chemotherapy for WM.
  • BTK inhibitors represent a significant advancement in WM treatment.
  • Newer agents like BCL2 inhibitors and non-covalent BTK inhibitors show promise in relapsed/refractory settings.

Conclusions:

  • Contemporary WM management is becoming more nuanced with targeted therapies.
  • BTK inhibitors and other novel agents are expanding treatment options.
  • Further research, including comparative trials, is needed to optimize WM treatment strategies.