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Review
. 2018 Jan 1;10(1):e2018004.
doi: 10.4084/MJHID.2018.004. eCollection 2018.

Waldenstrom's Macroglobulinemia: An Update

Maddalena Mazzucchelli  1 , Anna Maria Frustaci  1 , Marina Deodato  1 , Roberto Cairoli  1 , Alessandra Tedeschi  1
Affiliations
Review

Waldenstrom's Macroglobulinemia: An Update

Maddalena Mazzucchelli et al. Mediterr J Hematol Infect Dis. .

Abstract

Waldenstrom Macroglobulinemia is a rare lymphoproliferative disorder with distinctive clinical features. Diagnostic and prognostic characterisation in WM significantly changed with the discovery of two molecular markers: MYD88 and CXCR4. Mutational status of these latter influences both clinical presentation and prognosis and demonstrated therapeutic implications. Treatment choice in Waldenstrom disease is strictly guided by patients age and characteristics, specific goals of therapy, the necessity for rapid disease control, the risk of treatment-related neuropathy, disease features, the risk of immunosuppression or secondary malignancies and potential for future autologous stem cell transplantation. The therapeutic landscape has expanded during the last years and the approval of ibrutinib, the first drug approved for Waldenstrom Macroglobulinemia, represents a significant step forward for a better management of the disease.

Keywords: Ibrutinib; Macroglobulinemia; Treatment-related Neuropathy; Waldenstrom Disease.

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Conflict of interest statement

Competing interests: The authors have declared that no competing interests exist.

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