SHM Converge Scientific Abstract ePoster Gallery-A Sickling Basis for Cholestasis

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This case describes severe cholestasis in a 23-year-old man with hemoglobin SS sickle cell disease and transfusion-related iron overload. He presented in a sickle cell pain crisis with marked jaundice, scleral icterus, and rapidly rising bilirubin, eventually becoming unmeasurable and reaching about 60 mg/dL. Ultrasound showed gallstones with only minimal ductal dilation, suggesting the liver injury was not primarily due to obstruction.<br /><br />The report explains that liver disease is common in sickle cell disease. During a pain crisis, red cell breakdown can cause hyperbilirubinemia, but sickling inside the liver can also obstruct sinusoids, reduce oxygen delivery to hepatocytes, and lead to intrahepatic cholestasis. This can progress to liver failure or even renal failure.<br /><br />Initial management is supportive care, but if liver tests and bilirubin do not improve, hematology should be consulted early. In this case, red cell exchange did not help, so plasma exchange transfusion (PLEX) was performed twice. This led to temporary improvement but then rebound hyperbilirubinemia. Hepatology evaluated him for transplant, but he was not a candidate. Eventually, after ongoing PLEX and supportive treatment, his bilirubin stabilized and then declined rapidly.<br /><br />He was discharged on folic acid, deferiprone, and ursodiol, and follow-up labs continued to show improvement. The case emphasizes the importance of early recognition of sickle cell-related cholestasis, close monitoring of bilirubin, transaminases, and INR, and early escalation to PLEX and possibly liver transplant when supportive care fails.

Asset Subtitle

Jay Kachoria

Meta Tag

Author List Jay Kachoria

Category Clinical Vignettes

Concept Sickle Cell Disease

Concept Cholestasis

Concept Liver Injury

Concept Bilirubin

Concept Supportive Care

Distinguished Non-Finalist

Presenter Organization Duke University

Presenting Author Jay Kachoria

Track Adult

Keywords

sickle cell disease

intrahepatic cholestasis

severe hyperbilirubinemia

pain crisis

plasma exchange transfusion

red cell exchange

liver failure

transfusion-related iron overload

gallstones

bilirubin monitoring

Sickle Cell Disease

Cholestasis

Liver Injury

Bilirubin

Supportive Care