Cutting the cord from myelodysplastic syndromes. Issue 2 (March 2015)

• Record Type: Journal Article
• Authors: Padron, Eric; Steensma, David P.
• Is Part Of: Current opinion in hematology. Volume 22:Issue 2(2015:Mar.)
• Publication Date: 2015-03
• Subjects: Hematology -- Periodicals; Blood -- Diseases -- Periodicals; 616.15
• DOI: 10.1097/MOH.0000000000000112
• Languages: English
• ISSNs: 1065-6251

Abstract

<abstract> <title> <x xml:space="preserve">Abstract</x> </title> <sec> <title>Purpose of review</title> <p>Chronic myelomonocytic leukemia (CMML) is a troublesome hematologic malignancy characterized by peripheral blood monocytosis, marrow dysplasia, cytopenias, frequent extramedullary involvement by clonal cells, and a propensity for progression to acute myeloid leukemia. Although previously considered a subtype of the myelodysplastic syndromes (MDS), CMML is now recognized as a distinct entity with unique biologic and clinical features. This change has created a scientific and clinical research landscape that makes it difficult to discern CMML-specific validated conclusions versus speculative extrapolation from more general MDS data.</p> </sec> <sec> <title>Recent findings</title> <p>Here, we review recent biologic observations that support the current CMML WHO classification, such as the high frequency of <italic>SRSF2</italic> and <italic>ASXL1</italic> mutations compared with MDS and critical dependence of CMML cells on granulocyte–macrophage colony-stimulating factor signaling. In addition, we discuss CMML-specific prognostic tools and therapeutic results of agents developed for MDS in patients with CMML.</p> </sec> <sec> <title>Summary</title> <p>The present review focuses on evidence supporting CMML ontology and identifies key clinical differences in the management of CMML and that of MDS subtypes.</p> </sec> </abstract>