Long‐term outcome of liver transplant patients with Budd‐Chiari syndrome secondary to myeloproliferative neoplasms

• DOI: 10.1111/liv.12816
• Publication Date: 2015-03-03T14:26:53Z
• Authors: Andrej Potthoff; Dina Attia; Sven Pischke; Ingmar Mederacke; Gernot Beutel; Kinan Rifai; Katja Deterding; Karlheinz Heiring...; Jürgen Klempnauer; Christian P. Stra...; Michael P. Manns; Matthias J. Bahr
• Keywords: Budd–Chiari syndrome; Myeloproliferative Disorders

A considerable proportion of patients receiving liver transplants for Budd-Chiari syndrome (BCS) suffer from myeloproliferative neoplasms (MPN). This study evaluated the long-term prognosis liver-transplanted with BCS secondary to MPN and effect immunosuppression on progression.A total 78 were between 1982 2013. Of those, 40 suffered polycythaemia vera (PV) essential thrombocythaemia (ET). One patient had primary myelofibrosis (PMF). All received standard immunosuppressive regimen. We retrospectively survival, clinical course laboratory parameters MPN.Exactly 29/41 (71%) survived ≥ 3 years [mean age 36 ± 11 years; females n = 27 (93%)]. Mean follow-up after orthotopic transplantation (OLT) was 12.4 7.3 (range 3-28 years). Five- 10-year survival rates not significantly different in without (P 0.81 P 0.66 respectively) or PV ET 0.29 0.55 respectively). Thrombosis bleeding developed 7/29 (24%) survivors no significant difference 0.18). In follow-up, there evidence progression overt acute myeloid leukaemia (AML). uni- multivariate Cox-regression analyses, did influence OLT.Budd-Chiari underlying similar OLT. There enhanced OLT therapy. However, major haemorrhage recurrent thrombosis contributed morbidity mortality those patients.