Myelodysplastic/myeloproliferative neoplasms (MDS/MPN) are hybrid group of chronic myeloid neoplasms combining features of both MDS and MPN. The World Health Organization classification coined this group designation in 2008 to include chronic myelomonocytic leukemia, atypical chronic myeloid leukemia, juvenile myelomoncoytic leukemia, refractory anemia with ring sideroblasts and thrombocytosis as a provisional entity, and MDS/MPN unclassified. In this review, we highlight the challenges in diagnosing this group of the diseases, summarize the updates in classification, and discuss recent evolving understanding of the genetic landscape. We review risk-stratification models and overview the current management largely adapted from current MDS or MPN therapies. We define clinical benefit of therapy based on new proposed response criteria developed specifically for these groups of neoplasms. Finally, we introduce future opportunities including the planned international ABN MARRO (A Novel therapy combinations in untreated MDS/MPN And Relapsed/Refractory Overlap Syndromes) clinical study led by the MDS/MPN International Working Group.
Keywords: Atypical; CML; Chronic myelomonocytic leukemia; Myelodysplastic syndromes; Myeloproliferative neoplasms; Refractory anemia with ring sideroblasts and thrombocytosis.
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