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


METHODS
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.


RESULTS
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 long-term follow-up, there was no evidence of progression to overt myelofibrosis or acute myeloid leukaemia (AML). In the uni- and multivariate Cox-regression analyses, MPN did not influence survival after OLT.


CONCLUSIONS
Budd-Chiari syndrome patients with and without underlying MPN had similar long-term survival rates after OLT. There was no evidence of enhanced progression of MPN after OLT secondary to immunosuppressive therapy. However, major haemorrhage and recurrent thrombosis contributed to morbidity and mortality after OLT in those patients.
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
Andrej
A
Potthoff
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.|Department of Gastroenterology, Hepatology and Endemic Medicine, Beni Suef University, Beni Suef, Egypt.
Dina
D
Attia
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
Sven
S
Pischke
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
Ingmar
I
Mederacke
Department of Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany.
Gernot
G
Beutel
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
Kinan
K
Rifai
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
Katja
K
Deterding
Department of Visceral and Transplantation Surgery, Hannover Medical School, Hannover, Germany.
Karlheinz
K
Heiringhoff
Department of Visceral and Transplantation Surgery, Hannover Medical School, Hannover, Germany.
Jürgen
J
Klempnauer
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
Christian P
CP
Strassburg
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
Michael P
MP
Manns
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
Matthias J
MJ
Bahr
D000328:Adult; D006502:Budd-Chiari Syndrome / Q000150:complications* / Q000401:mortality / Q000601:surgery; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006084:Graft Rejection; D006085:Graft Survival; D019337:Hematologic Neoplasms / Q000150:complications* / Q000401:mortality / Q000601:surgery; D006801:Humans; D053208:Kaplan-Meier Estimate; D017093:Liver Failure / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009196:Myeloproliferative Disorders / Q000150:complications* / Q000401:mortality / Q000601:surgery; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018570:Risk Assessment; D015996:Survival Rate; D013997:Time Factors
Budd-Chiari syndrome; essential thrombocythaemia; liver transplantation; polycythaemia vera; primary myelofibrosis