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International Journal of Hematology
Published in: International Journal of Hematology 1/2017

01-01-2017 | Original Article

Disease characteristics and prognosis of myelodysplastic syndrome presenting with isolated thrombocytopenia

Authors: Julie Waisbren, Shira Dinner, Jessica Altman, Olga Frankfurt, Irena Helenowski, Juehua Gao, Brandon J. McMahon, Brady L. Stein

Published in: International Journal of Hematology | Issue 1/2017

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Abstract

Patients with myelodysplastic syndrome (MDS) who present with isolated thrombocytopenia (TCP) constitute a poorly described subgroup. The aim of the present study was to retrospectively evaluate disease characteristics and prognosis in patients with MDS and isolated TCP at a tertiary care center. Fifty patients (12 %) had isolated thrombocytopenia as the first presentation of MDS. Patients had varying MDS sub-classifications and cytogenetic profiles. The most common IPSS-R risk score was low (n = 24), although half of the patients had either IPSS-R intermediate (n = 18), high or very high risk disease (n = 7). Leukemic transformation occurred in 10 patients and there were 14 deaths (28 %) amongst all IPSS-R risk scores. Therapeutic agents used in this patient subgroup included hypomethylating agents and thrombopoietin receptor agonists. Overall, MDS with isolated TCP did not appear to have an inherently indolent course, as has been suggested previously. Future studies are needed to improve risk stratification, identify relevant contributors to disease pathogenesis, and better define treatment modalities.
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Metadata
Title
Disease characteristics and prognosis of myelodysplastic syndrome presenting with isolated thrombocytopenia
Authors
Julie Waisbren
Shira Dinner
Jessica Altman
Olga Frankfurt
Irena Helenowski
Juehua Gao
Brandon J. McMahon
Brady L. Stein
Publication date
01-01-2017
Publisher
Springer Japan
Published in
International Journal of Hematology / Issue 1/2017
Print ISSN: 0925-5710
Electronic ISSN: 1865-3774
DOI
https://doi.org/10.1007/s12185-016-2081-4

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