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Eur J Haematol. 2018 Mar;100(3):257-263. doi: 10.1111/ejh.13005. Epub 2018 Jan 17.

Significance of thrombocytopenia in patients with primary and postessential thrombocythemia/polycythemia vera myelofibrosis.

Author information

1
Department of Leukemia, University of Texas MD Anderson Cancer Center, Houston, TX, USA.
2
Kuwait Cancer Control Center, Shuwaikh, Kuwait.

Abstract

Severe thrombocytopenia (platelets <50 × 109 /L) is associated with very poor outcome of patients with myelofibrosis (MF). As patients with primary myelofibrosis (PMF) differ from patients with postessential thrombocythemia (PET-MF) and postpolycythemia vera myelofibrosis (PPV-MF), we aimed to evaluate the significance of low platelets among these patients. We present clinical characteristics and outcome of patients with either PMF, PPV-MF, or PET-MF, and thrombocytopenia who presented to our institution between 1984 and 2015. Of 1269 patients (877 PMF, 212 PPV-MF, 180 PET-MF), 11% and 14% had platelets either <50 × 109 /L or between 50-100 × 109 /L, respectively. Patients with platelets <50 × 109 /L were most anemic and transfusion dependent, had highest blast count and unfavorable karyotype. In general, their overall and leukemia-free survival was the shortest with median time of 15 and 13 months, respectively; with incidence of acute leukemia almost twice as high as in the remaining patients (6.9 vs 3.6 cases per 100 person-years). Nevertheless, this observation remains mostly significant for patients with PMF, as those with PEV/PVT-MF have already significantly inferior prognosis with platelets <100 × 109 /L.

KEYWORDS:

outcome; postessential thrombocythemia myelofibrosis; postpolycythemia vera myelofibrosis; primary myelofibrosis; thrombocytopenia

PMID:
29226426
PMCID:
PMC5814342
DOI:
10.1111/ejh.13005
[Indexed for MEDLINE]
Free PMC Article

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