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An Exercise in Extrapolation: Clinical Management of Atypical CML, MDS/MPN-Unclassifiable, and MDS/MPN-RS-T

  • Myelodysplastic Syndromes (D Steensma, Section Editor)
  • Published:
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Abstract

According to the recently published 2016 World Health Organization (WHO) classification of myeloid malignancies, myelodysplastic/myeloproliferative neoplasms (MDS/MPN) include atypical chronic myeloid leukemia (aCML), MDS/MPN-unclassifiable (MDS/MPN-U), chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia (JMML), and MDS/MPN ring sideroblasts with thrombocytosis (MDS/MPN-RS-T). MDS/MPN-RS-T was previously a provisional category known as refractory anemia with ring sideroblasts with thrombocytosis (RARS-T) which has now attained a distinct designation in the 2016 WHO classification. In this review, we focus on biology and management of aCML, MDS/MPN-U, and MDS/MPN-RS-T. There is considerable overlap between these entities which we attempt to further elucidate in this review. We also discuss recent advances in the field of molecular landscape that further defines and characterizes this heterogeneous group of disorders. The paucity of clinical trials available secondary to unclear pathogenesis and rarity of these diseases makes the management of these entities clinically challenging. This review summarizes some of the current knowledge of the molecular pathogenesis and suggested treatment guidelines based on the available data.

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Authors and Affiliations

  1. H. Lee Moffitt Cancer Center and Research Institute, Malignant Hematology, Tampa, FL, USA

    Chetasi Talati & Eric Padron

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  1. Chetasi Talati
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  2. Eric Padron
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Correspondence to Eric Padron.

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Chetasi Talati and Eric Padron each declare no potential conflict of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on Myelodysplastic Syndromes

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Talati, C., Padron, E. An Exercise in Extrapolation: Clinical Management of Atypical CML, MDS/MPN-Unclassifiable, and MDS/MPN-RS-T. Curr Hematol Malig Rep 11, 425–433 (2016). https://doi.org/10.1007/s11899-016-0350-1

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  • DOI: https://doi.org/10.1007/s11899-016-0350-1

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