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Wild-type Blocking PCR Combined with Direct Sequencing as a Highly Sensitive Method for Detection of Low-Frequency Somatic Mutations
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[Waldenström's macroglobulinemia. Current developments in diagnostics and therapy].

A Grunenberg1, C Buske2,3

  • 1Klinik für Innere Medizin III, Universitätsklinikum Ulm, Ulm, Deutschland.

Der Internist
|February 3, 2016
PubMed
Summary

Waldenström's macroglobulinemia (WM) is a rare B-cell lymphoma. Genetic subtypes exist, and while rituximab plus chemotherapy is standard, new treatments like ibrutinib offer chemotherapy-free options.

Keywords:
BortezomibIbrutinibMYD88 proteinRituximabWaldenstrom macroglobulinemiachemotherapy

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Area of Science:

  • Hematology
  • Oncology
  • Genetics

Background:

  • Waldenström's macroglobulinemia (WM) is a rare B-cell lymphoma, accounting for 1-2% of hematological neoplasms.
  • Genetic characterization enables the subdivision of WM into distinct genotypes.
  • Current treatment mainstays include the combination of rituximab and chemotherapy.

Purpose of the Study:

  • To review the current understanding of Waldenström's macroglobulinemia.
  • To discuss established and emerging therapeutic strategies for WM.
  • To highlight novel treatment modalities offering chemotherapy-free options.

Main Methods:

  • Literature review of Waldenström's macroglobulinemia.
  • Analysis of genetic classifications and their clinical relevance.
  • Evaluation of treatment outcomes for various therapeutic agents.

Main Results:

  • WM exhibits genetic heterogeneity, influencing disease classification.
  • Rituximab combined with chemotherapy remains a primary treatment approach.
  • Bortezomib shows efficacy as a proteasome inhibitor treatment.
  • Ibrutinib, an oral Bruton's tyrosine kinase inhibitor, presents a novel chemotherapy-free therapeutic option.

Conclusions:

  • Genetic insights are crucial for understanding WM subtypes.
  • Established treatments like rituximab-chemotherapy combinations are effective.
  • Emerging therapies, including ibrutinib, offer promising chemotherapy-free treatment avenues for WM patients.