Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 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

Lucia Masarova  1 , Ahmad Alhuraiji  2 , Prithviraj Bose  1 , Naval Daver  1 , Naveen Pemmaraju  1 , Jorge Cortes  1 , Sherry Pierce  1 , Hagop Kantarjian  1 , Srdan Verstovsek  1
Affiliations

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

Lucia Masarova et al. Eur J Haematol. 2018 Mar.

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.

PubMed Disclaimer

Conflict of interest statement

Disclosure of conflicts of interest: None.

Figures

Figure 1
Figure 1
Figure 2
Figure 2
Figure 3
Figure 3
See this image and copyright information in PMC

References

    1. Mesa RA, Silverstein MN, Jacobsen SJ, Wollan PC, Tefferi A. Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: an Olmsted County Study, 1976-1995. American journal of hematology. 1999;61(1):10–5. - PubMed
    1. Reilly JT, McMullin MF, Beer PA, Butt N, Conneally E, Duncombe A, et al. Guideline for the diagnosis and management of myelofibrosis. Br J Haematol. 2012;158(4):453–71. - PubMed
    1. Smith RE, Chelmowski MK, Szabo EJ. Myelofibrosis: a review of clinical and pathologic features and treatment. Critical reviews in oncology/hematology. 1990;10(4):305–14. - PubMed
    1. Lataillade JJ, Pierre-Louis O, Hasselbalch HC, Uzan G, Jasmin C, Martyre MC, et al. Does primary myelofibrosis involve a defective stem cell niche? From concept to evidence. Blood. 2008;112(8):3026–35. - PubMed
    1. Pikman Y, Lee BH, Mercher T, McDowell E, Ebert BL, Gozo M, et al. MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia. PLoS medicine. 2006;3(7):e270. - PMC - PubMed

MeSH terms