Acute Liver Failure With Liver Enzymes >5,000 in Sickle Cell Disease

Sickle cell disease is a hemoglobinopathy often complicated by painful vaso-occlusive episodes, acute chest syndrome, stroke, and myocardial infarction. Sickle cell intrahepatic cholestasis (SCIC) is a rare and potentially fatal complication of sickle cell disease. SCIC is thought to involve progres...

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Bibliographic Details
Published inACG case reports journal Vol. 11; no. 3; p. e01303
Main Authors Wadhavkar, Neha, Nsubuga, John Paul, Ibrahim, Nouran, Kumar, Prasanna, Hsu, Andrew, Simmons, Shannon
Format Journal Article
LanguageEnglish
Published United States Wolters Kluwer 01.03.2024
Subjects
Case Report
Liver
liver failure
transaminitis
sickle cell intrahepatic cholestasis
exchange transfusion
sickle cell disease
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Summary:Sickle cell disease is a hemoglobinopathy often complicated by painful vaso-occlusive episodes, acute chest syndrome, stroke, and myocardial infarction. Sickle cell intrahepatic cholestasis (SCIC) is a rare and potentially fatal complication of sickle cell disease. SCIC is thought to involve progressive hepatic injury due to sickling within sinusoids. We present the case of a young patient with SCIC and acute liver failure, requiring prompt treatment with exchange transfusion. Our case describes features that should raise suspicion for hepatic failure in SCIC and highlights exchange transfusion as a successful management approach in similar patients with an otherwise high risk of mortality.
ISSN:2326-3253
2326-3253
DOI:10.14309/crj.0000000000001303