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Novel Treatment Strategies in the Management of Waldenström Macroglobulinemia

  • Multiple Myeloma (P Kapoor, Section Editor)
  • Published:
Current Hematologic Malignancy Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Recent advances the genomic profiling of patients with Waldenström macroglobulinemia (WM) have led to the identification of novel therapeutic targets in these patients. In this review, we cover the current standard of care and the recently evaluated novel approaches with high potential to be incorporated in the therapeutic armamentarium against WM.

Recent Findings

The MYD88L265P mutation is the most common genomic abnormality in WM, and is encountered in 80–95% of patients, making it an important target for drug development. The success of the first-generation Bruton tyrosine kinase (BTK) inhibitor, ibrutinib, has generated tremendous interest in the study of more selective and potent BTK inhibitors. Additionally, the identification of CXCR4WHIM mutations in up to approximately 40% of patients with WM has fueled research regarding their implication on systemic therapy in WM.

Summary

In a rapidly advancing field of targeted therapies, the treatment options for patients with WM are expanding as researchers continue to uncover and harness the survival pathways active in this hematologic malignancy.

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Abbreviations

IR:

Ibrutinib and rituximab

MRR:

Major response rate (≥ partial response)

NA:

Not available

ORR:

Overall response rate

OS:

Overall survival

PFS:

Progression-free survival

R:

Rituximab

RR:

Relapsed refractory

TN:

Treatment naïve

CAR-T:

Chimeric antigen receptor

CLL:

Chronic lymphocytic leukemia

DRC:

Dexamethasone, rituximab, cyclophosphamide

PI3K:

Phosphoinositide 3-kinase

MCL:

Mantle cell lymphoma

MZL:

Marginal zone lymphoma

WM:

Waldenstrom macroglobulinemia

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Authors and Affiliations

  1. Department of Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Saurabh Zanwar, Jithma Prasad Abeykoon & Prashant Kapoor

  2. Division of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Jithma Prasad Abeykoon & Prashant Kapoor

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  1. Saurabh Zanwar
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  2. Jithma Prasad Abeykoon
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  3. Prashant Kapoor
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Correspondence to Prashant Kapoor.

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Zanwar, S., Abeykoon, J.P. & Kapoor, P. Novel Treatment Strategies in the Management of Waldenström Macroglobulinemia. Curr Hematol Malig Rep 15, 31–43 (2020). https://doi.org/10.1007/s11899-020-00559-4

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