Clinical features of patients with Philadelphia-negative myeloproliferative neoplasms complicated by portal hypertension

Clin Lymphoma Myeloma Leuk. 2015 Jan;15(1):e1-5. doi: 10.1016/j.clml.2014.04.004. Epub 2014 Jun 11.


Background: Portal hypertension has been reported to afflict 7% to 18% of patients with Philadelphia-negative MPNs, with complications of variceal bleeding and ascites. The clinical features and outcomes of these patients are unclear.

Patients and methods: In this multicenter retrospective study, we evaluated the clinical features of 51 patients with MPNs complicated by PHTN.

Results: The diagnosis of underlying MPN was most frequently PV (39%) and primary MF (35%), followed by post-PV MF (18%), ET (4%), and post-ET MF (4%). Frequency of Janus Kinase 2 V617F mutation appears as expected in the underlying MPN. Thrombosis within the splanchnic circulation was prevalent in patients with polycythemia compared with other MPNs (76% vs. 26%; P = .0007).

Conclusion: PV and MF patients have a greater incidence of PHTN in our population, with thrombosis contributing to PHTN development in PV patients. Patients with splanchnic circulation thrombosis are potential candidates for screening for PHTN. These data might be useful for developing screening strategies for early detection of PHTN in patients with MPN.

Keywords: Essential thromobocythemia; JAK inhibitor; Myelofibrosis; Polycythemia vera; Thrombosis.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Female
  • Follow-Up Studies
  • Humans
  • Hypertension, Portal / complications*
  • Hypertension, Portal / diagnosis*
  • Hypertension, Portal / therapy
  • Incidence
  • Janus Kinase 2 / antagonists & inhibitors
  • Janus Kinase 2 / genetics
  • Male
  • Middle Aged
  • Mutation
  • Myeloproliferative Disorders / complications*
  • Myeloproliferative Disorders / diagnosis*
  • Myeloproliferative Disorders / genetics
  • Philadelphia Chromosome
  • Portasystemic Shunt, Transjugular Intrahepatic
  • Prognosis
  • Protein Kinase Inhibitors / therapeutic use
  • Retrospective Studies
  • Splanchnic Circulation
  • Thrombosis / epidemiology
  • Thrombosis / etiology
  • Treatment Outcome


  • Protein Kinase Inhibitors
  • Janus Kinase 2