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Myeloproliferative neoplasms and recurrent thrombotic events in patients undergoing liver transplantation for Budd-Chiari syndrome: a single-center experience.
Oldakowska-Jedynak, Urszula; Ziarkiewicz, Mateusz; Ziarkiewicz-Wróblewska, Bogna; Dwilewicz-Trojaczek, Jadwiga; Górnicka, Barbara; Nyckowski, Pawel; Paluszkiewicz, Rafal; Wróblewski, Tadeusz; Zieniewicz, Krzysztof; Patkowski, Waldemar; Paczek, Leszek; Jedrzejczak, Wieslaw Wiktor; Krawczyk, Marek.
Afiliación
  • Oldakowska-Jedynak U; Department of General, Transplantation and Liver Surgery, Medical Univeristy of Warsaw, Warsaw, Poland.
  • Ziarkiewicz M; Department of Hematology, Oncology and Internal Diseases, Medical University of Warsaw, Warsaw, Poland.
  • Ziarkiewicz-Wróblewska B; Department of Pathology, Center for Biostructure Research, Medical University of Warsaw, Warsaw, Poland.
  • Dwilewicz-Trojaczek J; Department of Hematology, Oncology and Internal Diseases, Medical University of Warsaw, Warsaw, Poland.
  • Górnicka B; Department of Pathology, Center for Biostructure Research, Medical University of Warsaw, Warsaw, Poland.
  • Nyckowski P; Department of General, Transplantation and Liver Surgery, Medical University of Warsaw, Warsaw, Poland.
  • Paluszkiewicz R; Department of General, Transplantation and Liver Surgery, Medical University of Warsaw, Warsaw, Poland.
  • Wróblewski T; Department of General, Transplantation and Liver Surgery, Medical University of Warsaw, Warsaw, Poland.
  • Zieniewicz K; Department of General, Transplantation and Liver Surgery, Medical University of Warsaw, Warsaw, Poland.
  • Patkowski W; Department of General, Transplantation and Liver Surgery, Medical University of Warsaw, Warsaw, Poland.
  • Paczek L; Department of Immunology, Transplantology and Internal Medicine, Medical University of Warsaw, Warsaw, Poland.
  • Jedrzejczak WW; Department of Hematology, Oncology and Internal Medicine, Medical University of Warsaw, Warsaw, Poland.
  • Krawczyk M; Department of General, Transplantation and Liver Surgery, Medical University of Warsaw, Warsaw, Poland.
Ann Transplant ; 19: 591-7, 2014 Nov 14.
Article en En | MEDLINE | ID: mdl-25394736
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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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Trombosis / Trasplante de Hígado / Síndrome de Budd-Chiari / Trastornos Mieloproliferativos Tipo de estudio: Etiology_studies / Evaluation_studies / Guideline / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Adult / Female / Humans / Male / Middle aged Idioma: En Revista: Ann Transplant Asunto de la revista: TRANSPLANTE Año: 2014 Tipo del documento: Article País de afiliación: Polonia Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Trombosis / Trasplante de Hígado / Síndrome de Budd-Chiari / Trastornos Mieloproliferativos Tipo de estudio: Etiology_studies / Evaluation_studies / Guideline / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Adult / Female / Humans / Male / Middle aged Idioma: En Revista: Ann Transplant Asunto de la revista: TRANSPLANTE Año: 2014 Tipo del documento: Article País de afiliación: Polonia Pais de publicación: Estados Unidos