Splanchnic vein thrombosis in myeloproliferative neoplasms: Risk factors for recurrences in a cohort of 181 patients

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We retrospectively studied 181 patients with polycythaemia vera (n=67), essential thrombocythaemia (n=67) or primary myelofibrosis (n=47), who presented a first episode of splanchnic vein thrombosis (SVT). Budd-Chiari syndrome (BCS) and portal vein thrombosis were diagnosed in 31 (17.1%) and 109 (60.3%) patients, respectively; isolated thrombosis of the mesenteric or splenic veins was detected in 18 and 23 cases, respectively. After this index event, the patients were followed for 735 patient years (pt-years) and experienced 31 recurrences corresponding to an incidence rate of 4.2 per 100 pt-years. Factors associated with a significantly higher risk of recurrence were BCS (hazard ratio (HR): 3.03), history of previous thrombosis (HR: 3.62), splenomegaly (HR: 2.66) and leukocytosis (HR: 2.8). Vitamin K-antagonists (VKA) were prescribed in 85% of patients and the recurrence rate was 3.9 per 100 pt-years, whereas in the small fraction (15%) not receiving VKA more recurrences (7.2 per 100 pt-years) were reported. Intracranial and extracranial major bleeding was recorded mainly in patients on VKA and the corresponding rate was 2.0 per 100 pt-years. In conclusion, despite anticoagulation treatment, the recurrence rate after SVT in myeloproliferative neoplasms is high and suggests the exploration of new avenues of secondary prophylaxis with new antithrombotic drugs and JAK-2 inhibitors.


  • V. De Stefano
  • A. M. Vannucchi
  • M. Ruggeri
  • F. Cervantes
  • A. Alvarez-Larrán
  • A. Iurlo
  • M. L. Randi
  • L. Pieri
  • E. Rossi
  • P. Guglielmelli
  • S. Betti
  • E. Elli
  • M. C. Finazzi
  • G. Finazzi
  • N. Vianelli
  • G. Gaidano
  • I. Nichele
  • D. Cattaneo
  • M. Palova
  • M. H. Ellis
  • E. Cacciola
  • A. Tieghi
  • J. C. Hernandez-Boluda
  • E. Pungolino
  • G. Specchia
  • D. Rapezzi
  • A. Forcina
  • C. Musolino
  • A. Carobbio
  • M. Griesshammer
  • T. Barbui
Enheter & grupper
Externa organisationer
  • Catholic University of the Sacred Heart
  • University of Florence
  • Ospedale San Bortolo
  • Institutd' Investigacions Biomèdiques August Pi iSunyer (IDIBAPS)
  • Hospital del Mar Medical Research Institute
  • Maggiore Hospital Policlinico
  • University of Padova
  • University of Milano-Bicocca
  • Ospedale Papa Giovanni XXIII
  • Skåne University Hospital
  • St. Orsola-Malpighi University Hospital
  • University of Eastern Piedmont
  • University Hospital of Olomouc
  • Institute Meir Medical Center
  • University of Catania
  • Arcispedale Santa Maria Nuova
  • University and Polytechnic Hospital la Fe
  • Niguarda Hospital
  • Bari University Hospital
  • A.O. Santa Croce e Carle
  • San Raffaele Scientific Institute
  • Ruhr-University Bochum

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Hematologi
TidskriftBlood Cancer Journal
StatusPublished - 2016 nov 4
Peer review utfördJa