Sickle Cell Anemia Research Today is a free monthly online journal that collates and summarizes the latest research about Sickle Cell Anemia, including details on genetics, causes, symptoms.

Liver transplantation in children with sickle-cell disease.

Mekeel KL, Langham MR, Gonzalez-Peralta R, Fujita S, Hemming AW

Division of Transplantation, Department of Surgery, University of Florida, Gainesville, FL 32610, USA.

Severe liver disease is an unusual but potentially fatal complication of sickle-cell disease (SCD). Liver transplantation has been complicated by ongoing SCD and thrombosis. We reviewed 214 pediatric transplants done at our institution from 1990 to 2005. Three patients were transplanted for complications of SCD, including intrahepatic cholestasis and viral hepatitis. Overall patient and graft survival was 66%. One patient died after 6 years from a subdural hematoma. There were not any incidences of graft loss, primary nonfunction, or thrombosis. All 3 patients required between 1 and 4 postoperative transfusions to keep hemoglobin (Hgb) >9 g/dL with an S fraction of less than 25%. One patient required a preoperative transfusion for a hemoglobin S (HbS) fraction of 30%. Mean follow-up has been 4.2 years (range, 2.6-5.4 years). All 3 children continued to suffer sequelae from their SCD. One child suffered from recurrent sickle-cell hepatopathy and chronic graft failure. In conclusion, children with SCD can in rare instances develop acute and chronic liver failure. These children can be successfully transplanted with good outcomes. Careful attention must be paid to HbS fraction and hemoglobin level to prevent sickling and vascular thrombosis. Unfortunately, liver transplant cannot alter the natural course of the disease.

Published 16 April 2007 in Liver Transpl, 13(4): 505-8.
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