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Updated: Jun 2, 2026
05:23
Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Intrahepatic cholestasis in sickle cell disease: a case report
Denise Menezes Brunetta1, Ana Cristina Silva-Pinto, Maria do Carmo Favarin de Macedo
1Hematology 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
|April 15, 2011
Summary
Severe sickle cell intrahepatic cholestasis (SCIC) is a rare, fatal complication of sickle cell disease (SCD). Aggressive exchange transfusion (ET) successfully treated a severe SCIC case, leading to complete hepatic recovery.
Area of Science:
- Hematology
- Hepatology
- Genetics
Background:
- Sickle cell disease (SCD) is a genetic blood disorder.
- Severe sickle cell intrahepatic cholestasis (SCIC) is a rare but life-threatening complication of SCD, predominantly in homozygous sickle cell anemia patients.
- SCIC presents with hepatic failure, hyperbilirubinemia, and coagulopathy, distinct from viral hepatitis or biliary obstruction.
Observation:
- A patient with severe SCIC presented with liver enlargement and signs of hepatic failure.
- Diagnostic workup excluded viral hepatitis and biliary obstruction.
- The patient's condition indicated severe hepatic dysfunction.
Findings:
- Aggressive manual exchange transfusion (ET) was administered to reduce HbS levels below 30%.
- Multiple ET sessions were performed successfully.
- The patient achieved complete recovery of liver function post-treatment.
Implications:
- Exchange transfusion (ET) is an effective therapeutic strategy for severe sickle cell intrahepatic cholestasis (SCIC).
- Early initiation of ET is recommended for managing this severe SCD complication.
- This case highlights ET as a critical intervention for SCIC, improving patient outcomes.
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