Brunetta Denise Menezes, Silva-Pinto Ana Cristina, do Carmo Favarin de Macedo Maria, Bassi Sarah Cristina, Piccolo Feliciano Joao Victor, Ribeiro Fernanda Borges, Prado Benedito de Pina Almeida, De Santis Gil Cunha, de Lucena Angulo Ivan, Covas Dimas Tadeu
Hematology Division and Center for Cell Based Therapy, Department of Internal Medicine, Medical School of Ribeirao Preto, University of Sao Paulo, 14051-140 Ribeirao Preto, SP, Brazil.
Anemia. 2011;2011:975731. doi: 10.1155/2011/975731. Epub 2010 Dec 21.
Intrahepatic cholestasis (SCIC) is an uncommon but potentially fatal complication of sickle cell disease (SCD), with a high death rate, observed mainly in patients with homozygous sickle cell anemia. Herein, we describe a case of severe SCIC treated successfully with aggressive manual exchange transfusion (ET). The patient was admitted with enlarged liver and signs of hepatic failure, such as hyperbilirubinemia and coagulopathy. There was no evidence of viral hepatitis or biliary obstruction. We performed several sessions of ET in order to reduce the percentage of HbS to levels inferior to 30%, which was successfully accomplished. The patient had a complete recovery of hepatic function. This case has shown that ET is an effective treatment of SCIC and should be introduced early on the onset of this severe complication.
肝内胆汁淤积（SCIC）是镰状细胞病（SCD）一种罕见但可能致命的并发症，死亡率高，主要见于纯合子镰状细胞贫血患者。在此，我们描述一例通过积极的手工换血疗法（ET）成功治疗的严重SCIC病例。该患者因肝脏肿大及肝功能衰竭体征入院，如高胆红素血症和凝血障碍。无病毒性肝炎或胆道梗阻证据。我们进行了多次ET以将血红蛋白S（HbS）百分比降至30%以下，这一目标成功实现。患者肝功能完全恢复。该病例表明ET是SCIC的有效治疗方法，应在这一严重并发症发作早期采用。
