Current management of patients with chronic myelomonocytic leukemia

Abstract

Purpose of review: The present review focuses on the current management of patients with chronic myelomonocytic leukemia (CMML) and the most recent developments in the field.

Recent findings: CMML is a heterogeneous malignant myeloid disorder sharing features of myelodysplastic syndromes (MDS) and myeloproliferative neoplasms and characterized by peripheral blood monocytosis and increased risk of progression to acute leukemia. Its natural course is highly variable and use of CMML-specific prognostic scoring systems is strongly recommended for tailoring treatment. Multiple recent studies have showed that somatic mutations, which are almost always present have a relevant and independent impact on survival but lack a clear role in predicting the response to currently available drugs.

Summary: The incorporation of somatic mutations to prognostic scoring systems has improved the prediction of patients' outcomes. Current treatment for CMML remains unsatisfactory. Allogeneic hematopoietic cell transplantation is the only curative option but is applicable to a minority of patients. Usually higher-risk patients displaying MDS-like characteristics are treated with hypomethylating agents (HMAs), whereas those with myeloproliferative features generally receive hydroxyurea or HMAs but none of these drugs substantially modify the natural history of CMML. Newer therapies are clearly needed.