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Myeloproliferative Neoplasms: Thrombophilic Clonal Stem Cell Diseases

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Coagulation in Cancer

Part of the book series: Cancer Treatment and Research ((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].

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    Ayalew Tefferi

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    Tefferi, A. (2009). Myeloproliferative Neoplasms: Thrombophilic Clonal Stem Cell Diseases. In: Kwaan, H., Green, D. (eds) Coagulation in Cancer. Cancer Treatment and Research, vol 148. Springer, Boston, MA. https://doi.org/10.1007/978-0-387-79962-9_10

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