Background:Myeloproliferative neoplasms (MPNs) are frequently identified as an underlying cause in patients with non-cirrhotic portal vein thrombosis (PVT). The aim of this study was to describe the long-term outcome of patients with PVT and MPN. Methods:A cohort study was performed including all adult patients referred to our hospital between 1980 and 2008 with non-cirrhotic, non-malignant PVT and confirmed MPN. Results:A total of 44 patients (70% female) were included, with a median age at PVT-diagnosis of 48years (range 18-79). In 31 patients (70%) PVT was the first manifestation of an MPN. Additional risk factors for thrombosis were present in 20 patients (45%). Median follow-up was 5.8years (range 0.4-21). Twenty-three patients (52%) were treated with oral anticoagulants after diagnosis of PVT, of whom 15 (34%) received long-term therapy. During follow-up, 17 patients (39%) experienced at least one episode of gastrointestinal bleeding. Additional thrombotic events occurred in 12 patients (27%). Twelve patients (27%) had progression of the underlying MPN. Seventeen patients (39%) died at a median age of 64years (range 30-88). Death was directly related to end-stage MPN in eight patients (47%) and to a new thrombotic event in three patients (18%). No patients died from gastrointestinal bleeding. Conclusions:PVT is often the presenting symptom of an underlying MPN, highlighting the need for thorough screening for this disease. Recurrent thrombosis is a common and severe complication in patients with PVT and MPN. Mortality is primarily related to the underlying MPN and not to complications of portal hypertension.

Antiplatelet drugs, Myeloproliferative neoplasm, Portal vein thrombosis, Venous thrombosis, Vitamin K antagonists,
Journal of Thrombosis and Haemostasis
Erasmus MC: University Medical Center Rotterdam

Hoekstra, J, Bresser, E.L, Smalberg, J.H, Spaander, M.C.W, Leebeek, F.W.G, & Janssen, H.L.A. (2011). Long-term follow-up of patients with portal vein thrombosis and myeloproliferative neoplasms. Journal of Thrombosis and Haemostasis, 9(11), 2208–2214. doi:10.1111/j.1538-7836.2011.04484.x