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.

Can exchange transfusions treat postoperative intrahepatic colestasis in patients with sickle cell anemia?

Delis SG, Touloumis Z, Bourli A, Madariaga J, Dervenis C

Liver Surgical Unit, Agia Olga Hospital, Athens, Greece, and Department of Surgery, University of Miami, FL, USA.

BACKGROUND: Although the most common cause of liver failure (LF) in hematologic patients is viral hepatitis, several episodes of sickle cell intrahepatic cholestasis (IHC) have been reported as rare but potentially causative of fulminant LF. Reviewing the literature, we have presented a single case of intrahepatic cholestasis after major liver resection, which was effectively treated by exchange transfusion. METHODS: Serial hemoglobin S, D levels and liver enzymes were monitored postoperatively. RESULTS: Although the patient's intra- and postoperative courses were uneventful, an increased serum bilirubin was identified to be due to intrahepatic sinusoid congestion and subsequent cholestasis. Exchange transfusion was required to maintain HbS below 20% and reverse bilirubin levels to normal values. CONCLUSION: Sickle cell anemia is a rare cause of cholestasis after major hepatic surgery. To our knowledge, this case is the only documented incidence of IHC following major hepatectomy that was effectively treated with exchange transfusion.

Published 26 June 2006 in Transplant Proc, 38(5): 1385-6.
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