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14 November 2014 : Original article  

Myeloproliferative Neoplasms and Recurrent Thrombotic Events in Patients Undergoing Liver Transplantation for Budd-Chiari Syndrome: A Single-Center Experience

Urszula Oldakowska-JedynakACDFG, Mateusz ZiarkiewiczBCEF, Bogna Ziarkiewicz-WróblewskaB, Jadwiga Dwilewicz-TrojaczekACD, Barbara GórnickaB, Paweł NyckowskiAD, Rafał PaluszkiewiczCD, Tadeusz WróblewskiAC, Krzysztof ZieniewiczAC, Waldemar PatkowskiAC, Leszek PączekD, Wiesław Wiktor JedrzejczakDE, Marek KrawczykDG

DOI: 10.12659/AOT.890834

Ann Transplant 2014; 19:591-597

Abstract

BACKGROUND: Budd-Chiari syndrome is a heterogeneous disease. The role of liver transplantation as a treatment option has been discussed since 1976. Many cases are related to underlying myeloproliferative neoplasms associated with prothrombotic propensity. The aim of this study was to evaluate the long-term clinical outcome after liver transplantation for Budd-Chiari syndrome at our center, with special emphasis on recurrent thrombosis and underlying myeloproliferative disorders.

MATERIAL AND METHODS: A medical records search revealed 25 patients transplanted at our center for Budd-Chiari syndrome between 2000 and 2009. Indications for transplantation were complications of end-stage liver disease or acute liver failure.

RESULTS: Ten patients were men (40.0%). Median age of recipients at transplantation was 29.0 (17–51) years. Eighteen patients (72%) had evidence of myeloproliferation, 1 had paroxysmal nocturnal hemoglobinuria, and 6 had idiopathic disease. In 55.5% of cases eventually diagnosed with myeloproliferative neoplasms, Budd-Chiari syndrome was their initial presentation. All patients were maintained on long-term post-transplant anticoagulation protocol. The median follow-up time was 58.8 months. Four patients (16%) died during follow-up. Acute graft rejection occurred in 16% of cases. During the observation period, 5 patients had recurrent thrombotic events. The 5-year patient and graft survival rate was 84%. No case of transformation to acute leukemia was seen.

CONCLUSIONS: Our data show satisfactory long-term survival of patients and grafts in the study group. Occult course of myeloproliferative neoplasms is frequent in this population and exceeds 50%. We observed recurrent thrombosis in 20% of recipients.

Keywords: Anticoagulants, Budd-Chiari syndrome, Liver Transplantation, myeloproliferative disorders

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Annals of Transplantation eISSN: 2329-0358
Annals of Transplantation eISSN: 2329-0358