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  • Title: Myeloproliferative neoplasms and recurrent thrombotic events in patients undergoing liver transplantation for Budd-Chiari syndrome: a single-center experience.
    Author: Oldakowska-Jedynak U, Ziarkiewicz M, Ziarkiewicz-Wróblewska B, Dwilewicz-Trojaczek J, Górnicka B, Nyckowski P, Paluszkiewicz R, Wróblewski T, Zieniewicz K, Patkowski W, Pączek L, Jedrzejczak WW, Krawczyk M.
    Journal: Ann Transplant; 2014 Nov 14; 19():591-7. PubMed ID: 25394736.
    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/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.
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