title: Long-term follow-up of patients with portal vein thrombosis and myeloproliferative neoplasms.
abstract: 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 48 years (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 5.8 years (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 64 years (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.
authors: Department of Gastroenterology and Hepatology, Erasmus MC, University Medical Center Rotterdam, Rotterdam.
authors: J
authors: J
authors: Hoekstra
authors: E L
authors: EL
authors: Bresser
authors: J H
authors: JH
authors: Smalberg
authors: M C W
authors: MC
authors: Spaander
authors: F W G
authors: FW
authors: Leebeek
authors: H L A
authors: HL
authors: Janssen
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000925:Anticoagulants / Q000627:therapeutic use; D002423:Cause of Death; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006470:Hemorrhage; D006801:Humans; D006975:Hypertension, Portal; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000150:complications* / Q000401:mortality; D011169:Portal Vein / Q000473:pathology; D012307:Risk Factors; D015996:Survival Rate; D016896:Treatment Outcome; D020246:Venous Thrombosis / Q000209:etiology* / Q000401:mortality; D055815:Young Adult
chemical_list: D000925:Anticoagulants