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.

A O Ospedale Niguarda Ca' Granda Milano Italy

A O Santa Croce e Carle Cuneo Italy

A O Universitaria Messina Italy

A O Universitaria Policlinico di Bari Italy

Arcispedale Santa Maria Nuova IRCCS Reggio Emilia Italy

Center for Research and Innovation of Myeloproliferative Neoplasms A O U Careggi University of Florence Florence Italy

Clinica Medica 1 Università di Padova Padova Italy

Department of Hematology Hospital del Mar IMIM Barcelona Spain

Department of Hematology Institute Meir Medical Center Kfar Saba Israel

Department of Translational Medicine Università del Piemonte Orientale Vercelli Italy

Divisione di Ematologia Ospedale San Gerardo ASST Monza Italy

FROM Research Foundation ASST Papa Giovanni XXIII Bergamo Italy

Haemostasis Unit Department of Medical Surgical and Advanced Technologies Sciences 'G F Ingrassia' University of Catania Catania Italy

Hematology Division ASST Papa Giovanni XXIII Bergamo Italy

Hospital Clínic IDIBAPS Barcelona Spain

Hospital Clinico Valencia Spain

Institute of Hematology and Medical Oncology S Orsola Malpighi Hospital University of Bologna Bologna Italy

Institute of Hematology Catholic University Roma Italy

IRCCS Ospedale San Raffaele Milano Italy

Johannes Wesling Medical Center Minden University of Bochum Minden Germany

Oncohematology Division Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milano Italy

Ospedale San Bortolo Vicenza Italy

Skane University Hospital Lund Sweden

University Hospital of Olomouc Olomouc Czech Republic

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