Myeloproliferative neoplasms and recurrent thrombotic events in patients undergoing liver transplantation for Budd-Chiari syndrome: a single-center experience.

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.

• Authors: 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
• Journal: Annals of transplantation (Ann Transplant) Vol. 19 Pg. 591-7 (Nov 14 2014) ISSN: 2329-0358 [Electronic] United States
• PMID: 25394736 (Publication Type: Evaluation Study, Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Adolescent
• Adult
• Budd-Chiari Syndrome (etiology, surgery)
• Female
• Follow-Up Studies
• Graft Rejection (epidemiology)
• Graft Survival
• Humans
• Liver Transplantation
• Male
• Middle Aged
• Myeloproliferative Disorders (complications)
• Postoperative Complications
• Recurrence
• Retrospective Studies
• Thrombosis (complications)
• Treatment Outcome
• Young Adult