Recurrence of Budd-Chiari syndrome after liver transplantation in paroxysmal nocturnal hemoglobinuria.
Venous thrombembolism is a major complication of paroxysmal nocturnal hemoglobinuria (PNH). Often, veins of atypical localization are afflicted, resulting in cerebral, mesenteric, or hepatic venous thrombosis. We present a patient who received an orthotopic liver graft for chronic Budd-Chiari syndrome in 1988. PNH was the only thrombophilic predisposition identified in this patient. After transplantation, he repeatedly suffered from hemorrhage. Subsequently, the patient discontinued prophylactic anticoagulation nearly 10 years after transplantation. Within 6 months Budd-Chiari syndrome recurred, but stabilized after anticoagulation therapy with low-molecular-weight heparin was reinstituted. The patient is clinically stable 14 years after receiving the liver graft. Eleven cases of relapsing Budd-Chiari syndrome have been reported in the literature. Of these, four patients suffered from PNH. All patients transplanted for PNH-associated Budd-Chiari syndrome in these reports suffered from either major bleeding or thrombosis. In conclusion, patients afflicted with PNH appear to be at high risk of incurring complications after liver transplantation.
Bahr
Matthias J
MJ
Department of Gastroenterology, Hepatology and Endocrinology, Hanover University Medical School, Carl-Neuberg-Strasse 1, 30623 Hanover, Germany. bahr.matthias@mh-hannover.de
Schubert
Jörg
J
Bleck
Jörg S
JS
Tietge
Uwe J F
UJ
Boozari
Bita
B
Schmidt
Reinhold E
RE
Klempnauer
Jürgen
J
Strassburg
Christian P
CP
Manns
Michael P
MP
D000328:Adult; D006502:Budd-Chiari Syndrome / Q000000981:diagnostic imaging / Q000209:etiology*; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications*; D006801:Humans; D008102:Liver Circulation; D016031:Liver Transplantation; D008297:Male; D011183:Postoperative Complications; D012008:Recurrence; D014463:Ultrasonography