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

Valerio De Stefano, Alessandro M. Vannucchi, Marco Ruggeri, Francisco Cervantes, Alberto Alvarez-Larrán, Alessandra Iurlo, M. L. Randi, Lisa Pieri, E. Rossi, Paola Guglielmelli, Silvia Betti, Elena Elli, Mariachiara Finazzi, Guido Finazzi, E. Zetterberg, Nicola Vianelli, Gianluca Gaidano, Ilaria Nichele, Daniele Cattaneo, M. PalovaM. H. Ellis, Emma Cacciola, Alessia Tieghi, Juan Carlos Hernández-Boluda, Esther Pungolino, Giorgina Specchia, Davide Rapezzi, A. Forcina, Caterina Musolino, Alessandra Carobbio, M. Griesshammer, Tiziano Barbui

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Article numbere493
JournalBlood Cancer Journal
Issue number11
Publication statusPublished - Nov 4 2016

ASJC Scopus subject areas

  • Hematology
  • Oncology


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