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Myelodysplastic syndrome

Chronic myelomonocytic leukemia diagnosis and management

Leukemia volume 35, pages 1552–1562 (2021)Cite this article

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Abstract

Chronic myelomonocytic leukemia (CMML) is a rare, heterogeneous myeloid malignancy classified as a myelodysplastic syndromes/myeloproliferative neoplasm (MDS/MPN) overlap syndrome by the World Health Organization (WHO). Its initial presentation can be incidental or associated with myelodysplastic or myeloproliferative symptoms and up to 20% of patients harbor a concurrent inflammatory or autoimmune condition. Persistent monocytosis is the hallmark of CMML but diagnosis can be challenging. Increased understanding of human monocyte subsets, chromosomal abnormalities, and somatic gene mutations have led to more accurate diagnosis and improved prognostication. A number of risk stratification systems have been developed and validated but using those that incorporate molecular information such as CMML Prognostic Scoring System (CPSS)-Mol, Mayo Molecular, and Groupe Francophone des Myelodysplasies (GFM) are preferred. Symptom-directed approaches forms the basis of CMML management. Outcomes vary substantially depending on risk ranging from observation for a number of years to rapidly progressive disease and acute myeloid leukemia (AML) transformation. Patients who are low risk but with symptoms from cytopenias or proliferative features such as splenomegaly may be treated with hypomethylating agents (HMAs) or cytoreductive therapy, respectively, with the goal of durable symptoms control. Allogeneic hematopoietic cell transplantation should be considered for intermediate to high risk patients. The lack of effective pharmaceutical options has generated interest in novel therapeutics for this disease, and early phase clinical trial results are promising.

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Fig. 1: The chronic myeloid neoplasms continuum.
Fig. 2: Recurrent gene mutations in CMML.
Fig. 3: CMML treatment algorithm.

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  1. Department of Malignant Hematology, Moffitt Cancer Center, Tampa, FL, USA

    Onyee Chan & Eric Padron

  2. INSERM U1287, Gustave Roussy Cancer Campus, Villejuif, France

    Aline Renneville

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O.C. wrote the original draft and made some of the figures. A.R. revised the manuscript and made some of the figures. E.P. revised the manuscript and supervised the work.

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EP: research funding from Incyte, Kura Oncology and BMS, honoraria from Novartis. The author declares no competing interests.

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Chan, O., Renneville, A. & Padron, E. Chronic myelomonocytic leukemia diagnosis and management. Leukemia 35, 1552–1562 (2021). https://doi.org/10.1038/s41375-021-01207-3

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