Myeloproliferative Neoplasms: Thrombophilic Clonal Stem Cell Diseases

  • Ayalew Tefferi
Part of the Cancer Treatment and Research book series (CTAR, volume 148)

Polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) are currently classified as BCR-ABL-negative classic myeloproliferative neoplasms (MPN). According to the revised 2008 World Health Organization (WHO) classification system, adult chronic myeloid neoplasms are now divided into four broad categories; myelodysplastic syndrome (MDS), MPN, “MDS/MPN overlap” and “myeloid or lymphoid neoplasms associated with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1” [1]. The WHO MPN category includes the four classic MPN: chronic myelogenous leukemia (CML; BCR-ABL-positive), PV, ET, and PMF. All three BCR-ABL-negative MPN are uncommon and incidence figures are estimated at 0.2–2.5/100,000 for ET [2–4], 0.4 to 1.5/100,000 for PMF [2, 3] and 0.8–2.6/100,000 for PV [5, 6]. The median age at diagnosis for all these three MPN is approximately 60 years [7].


Acute Myeloid Leukemia Polycythemia Vera Essential Thrombocythemia JAK2 Inhibitor Reduced Intensity Conditioning 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  1. 1.Division of Hematology, Mayo ClinicRochesterUS

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