title: Combined liver and kidney transplantation in a patient with sickle cell disease.
abstract: Sickle cell intrahepatic cholestasis is a potentially fatal end-organ complication of sickle cell anemia. Renal involvement in sickle cell anemia is common, and in some cases, can present as acute renal failure. Although renal transplants have been performed in patients with sickle cell anemia since the late 1960s and a number of liver transplants have been recently performed for these complications, there has not been experience with dual organ transplantation for sickle cell anemia-related complications. We describe the case of a patient with sickle cell anemia who underwent successful combined liver and kidney transplantation after the development of acute sickle cell intrahepatic cholestasis and renal failure requiring continuous venovenous hemodialysis. The patient underwent a successful combined liver and kidney transplant with limited perioperative complications and preserved allograft function. At 22 months posttransplant, the patient expired as a result of an acute pulmonary embolus in the setting of bilateral hip fractures. Autopsy revealed no evidence of liver or kidney allograft rejection and evidence of chronic sickle cell nephropathy in the native kidney. Combined liver and kidney transplantation is a viable therapeutic option in patients with severe end-organ effects of sickle cell anemia.
authors.affiliation: Medical (Gastrointestinal Unit) Services, Massachusetts General Hospital, Boston, Massachusetts 02114, USA.
authors.forename: Andrew S
authors.initials: AS
authors.lastname: Ross
authors.forename: Fiona
authors.initials: F
authors.lastname: Graeme-Cook
authors.forename: A Benedict
authors.initials: AB
authors.lastname: Cosimi
authors.forename: Raymond T
authors.initials: RT
authors.lastname: Chung
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology / Q000601:surgery; D001741:Black or African American; D000755:Anemia, Sickle Cell / Q000601:surgery*; D002779:Cholestasis / Q000209:etiology / Q000601:surgery; D017809:Fatal Outcome; D006801:Humans; D016030:Kidney Transplantation / Q000473:pathology; D016031:Liver Transplantation / Q000473:pathology; D008297:Male; D008404:Massachusetts; D008875:Middle Aged; D010146:Pain; D006435:Renal Dialysis; D014184:Transplantation, Homologous / Q000473:pathology