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Chronic Myelomonocytic Leukemia: 2018 Update to Prognosis and Treatment

  • Myeloproliferative Neoplasms (B Stein, Section Editor)
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Abstract

Purpose of Review

Chronic myelomonocytic leukemia (CMML) is a rare and often aggressive myeloid malignancy. Historically, prognostic markers and therapeutic paradigms have been applied from myelodysplastic syndromes (MDS) or myeloproliferative neoplasms (MPNs). Interest has increased recently in developing tailored approaches for the MDS/MPN overlap syndrome of CMML.

Recent Findings

Multiple prognostic scores have been validated specifically for CMML in the past 5 years. These incorporate somatic mutations, with ASXL1 mutations repeatedly correlating with poor prognosis. Accurate prognostication can guide treatment. Hypomethylating agents (HMAs) and curative allogeneic blood or marrow transplantation (BMT) remain the most available standard treatments. Recently, a number of novel approaches using unapproved therapies (i.e., lenalidomide, ruxolitinib, sotatercept, and tipifarnib) have demonstrated some efficacy in CMML.

Summary

Increased recognition and interest in CMML have led to the development of a number of new prognostic models and potential treatment options. Standard treatment options remain limited and clinical trials should be strongly considered whenever available.

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  1. Department of Blood and Marrow Transplantation and Cellular Immunotherapy, H. Lee Moffitt Cancer Center, Tampa, FL, USA

    Hany Elmariah

  2. Division of Hematologic Malignancies, Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, The Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Amy E. DeZern

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Elmariah, H., DeZern, A.E. Chronic Myelomonocytic Leukemia: 2018 Update to Prognosis and Treatment. Curr Hematol Malig Rep 14, 154–163 (2019). https://doi.org/10.1007/s11899-019-00509-9

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