Thrombosis in the Philadelphia Chromosome-Negative Myeloproliferative Neoplasms

Cancer Treat Res. 2019:179:159-178. doi: 10.1007/978-3-030-20315-3_11.


The myeloproliferative neoplasms (MPNs) are clonal stem cell-derived diseases. This chapter focuses on the subcategory of Philadelphia (Ph) chromosome-negative classical MPNs, polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF). These MPNs are associated with both microvascular and macrovascular thrombosis, which may occur in the venous and arterial circulation. Erythrocytosis, leukocytosis, and increased JAK2V617F allele burden are known to be risk factors. In this chapter, we review the thrombotic and hemostatic manifestations of the Philadelphia (Ph) chromosome-negative classical MPNs, including the clinical manifestations, the pathophysiology, as well as management.

Keywords: Essential thrombocythemia; JAK2V617F; Macrovascular thrombosis; Microvascular thrombosis; Myelofibrosis; Myeloproliferative neoplasms; Polycythemia vera.

Publication types

  • Review

MeSH terms

  • Bone Marrow Neoplasms / complications
  • Bone Marrow Neoplasms / genetics
  • Hemostasis / physiology
  • Humans
  • Mutation
  • Myeloproliferative Disorders / complications*
  • Myeloproliferative Disorders / genetics
  • Myeloproliferative Disorders / physiopathology
  • Philadelphia Chromosome
  • Polycythemia Vera / complications
  • Primary Myelofibrosis / complications
  • Thrombocythemia, Essential / complications
  • Thrombosis* / diagnosis
  • Thrombosis* / etiology
  • Thrombosis* / physiopathology
  • Thrombosis* / therapy