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

Liver International : Official Journal of the International Association for the Study of the Liver
Andrej PotthoffMatthias J Bahr

Abstract

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 of liver-transplanted patients with BCS secondary to MPN and the effect of immunosuppression on MPN progression. A total of 78 patients with BCS were evaluated between 1982 and 2013. Of those, 40 patients suffered from polycythaemia vera (PV) and essential thrombocythaemia (ET). One patient had primary myelofibrosis (PMF). All patients received the standard immunosuppressive regimen. We retrospectively evaluated the long-term survival, clinical course and laboratory parameters of patients with MPN. Exactly 29/41 patients (71%) with MPN survived ≥ 3 years [mean age 36 ± 11 years; females n = 27 (93%)]. Mean follow-up after orthotopic liver transplantation (OLT) was 12.4 ± 7.3 years (range 3-28 years). Five- and 10-year survival rates were not significantly different in patients with and without MPN (P = 0.81 and P = 0.66 respectively) or in patients with PV and ET (P = 0.29 and P = 0.55 respectively). Thrombosis and bleeding developed in 7/29 (24%) long-term MPN survivors with no significant difference between ET and PV (P = 0.18). In the lon...Continue Reading

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Jun 3, 2016·Liver International : Official Journal of the International Association for the Study of the Liver·Dhiraj TripathiSimon P Olliff
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Mar 20, 2015·Liver International : Official Journal of the International Association for the Study of the Liver·Andrea Mancuso
Apr 7, 2020·Clinics and Research in Hepatology and Gastroenterology·Audrey CoillyChristophe Bureau

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