Intrahepatic Cholestasis in Sickle Cell Disease: A Case Report
Denise Menezes Brunetta,
Ana Cristina Silva-Pinto,
Maria do Carmo Favarin de Macedo,
Sarah Cristina Bassi,
Joao Victor Piccolo Feliciano,
Fernanda Borges Ribeiro,
Benedito de Pina Almeida Prado,
Gil Cunha De Santis,
Ivan de Lucena Angulo,
Dimas Tadeu Covas
Affiliations
Denise Menezes Brunetta
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
Ana Cristina Silva-Pinto
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
Maria do Carmo Favarin de Macedo
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
Sarah Cristina Bassi
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
Joao Victor Piccolo Feliciano
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
Fernanda Borges Ribeiro
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
Benedito de Pina Almeida Prado
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
Gil Cunha De Santis
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
Ivan de Lucena Angulo
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
Dimas Tadeu Covas
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
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.
