Epistaxis: A Rare Presentation of Sickle Cell Intrahepatic Cholestasis
Boraan Abdulkarim1, Hannah Cushen2,3, James Grace2,3
1Department of Pediatrics, Loyola University Medical Center, Maywood, Illinois, USA.
Insights
Sickle cell hepatopathy (SCH) is a liver complication in sickle cell disease (SCD). This case highlights sickle cell intrahepatic cholestasis (SCIC) and its severe outcomes, emphasizing prompt management.
Area of Science:
- Hepatology
- Hematology
- Nephrology
Background:
- Sickle cell hepatopathy (SCH) encompasses liver disease in sickle cell disease (SCD).
- Sickle cell intrahepatic cholestasis (SCIC) is a rare but severe SCD complication.
- SCIC can lead to liver failure and necessitate transplantation.
Observation:
- A 24-year-old male with SCD presented with jaundice, encephalopathy, epistaxis, and pseudohematemesis.
- Clinical findings included hyperbilirubinemia, coagulopathy, portal hypertension, and acute kidney injury (AKI).
- Liver biopsy confirmed sinusoidal red cell sickling, fibrosis, and ductopenia consistent with SCIC.
Findings:
- Initial management with transfusions and apheresis was insufficient.
- The patient required liver transplantation due to ongoing complications.
- Transplantation was complicated by perihepatic hematoma and stroke.
Implications:
- This case underscores the critical need for early diagnosis of SCIC.
- Prompt, multidisciplinary management is essential to prevent liver failure.
- Timely intervention can reduce the likelihood of requiring liver transplantation.
Abstract:
Sickle cell hepatopathy (SCH) is an umbrella term relating to liver disease in sickle cell disease (SCD). This term ranges from common etiologies such as cholelithiasis to disease-specific causes such as sickle cell intrahepatic cholestasis (SCIC), a rare but significant complication of SCD capable of progressing to liver failure and consideration of transplantation. We report the case of a 24-year-old male with SCD who presented with jaundice, encephalopathy, uncontrollable epistaxis, and pseudohematemesis and was found to have hyperbilirubinemia, coagulopathy, portal hypertension, and acute kidney injury (AKI). This presentation was concerning for SCIC. Initial management included transfusions and a trial of apheresis. Liver biopsy revealed sinusoidal red cell sickling, fibrosis, and ductopenia, consistent with findings of SCIC. Due to ongoing complications, recurrent admissions, and symptomatic coagulopathy, the patient underwent liver transplantation which was complicated by perihepatic hematoma and stroke, necessitating extensive rehabilitation. This case emphasizes the importance of early diagnostic workup and prompt, multidisciplinary management of SCIC to mitigate risks of liver failure and need for transplant.
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