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Urszula Oldakowska-Jedynak, Mateusz Ziarkiewicz, Bogna Ziarkiewicz-Wróblewska, Jadwiga Dwilewicz-Trojaczek, Barbara Górnicka, Paweł Nyckowski, Rafał Paluszkiewicz, Tadeusz Wróblewski, Krzysztof Zieniewicz, Waldemar Patkowski, Leszek Pączek, Wiesław Wiktor Jedrzejczak, Marek Krawczyk
(Department of General, Transplantation and Liver Surgery, Medical Univeristy of Warsaw, Warsaw, Poland)
Ann Transplant 2014; 19:591-597
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.