S3161 Sickle Cell Hepatopathy: A Rare Complication of Sickle Cell Anemia

Abstract

Introduction: Sickle cell hepatopathy (SCH) describes the spectrum of hepatobiliary complications seen in sickle cell disease (SS). The etiology is multifaceted; repeated sickling causes liver vaso-occlusion and sinusoidal obstruction, which contributes to ischemic hepatic damage. SCH treatment is supportive; it includes supplemental oxygen, intravenous fluids and exchange transfusions. We present the diagnosis and management of a rare case of SCH. Case Description/Methods: Our patient is a 30-year-old male with SS anemia complicated by avascular necrosis of the shoulder, infrequent pain crises and cholecystectomy, who presented to our ED with fevers >100.4F, abdominal pain, acholic stools, dark urine and jaundice. Initial workup showed transaminitis, cholestasis, with an alkaline phosphatase 189, ALT 104, AST 145 and a total bilirubin 46.5. Abdominal ultrasound and CT did not show any acute hepatic causes for these lab abnormalities. Fevers prompted an infectious workup; viral hepatitis markers and blood cultures were negative. Autoimmune processes were ruled out with normal ANA, ASMA and immunoglobulins. He was given supportive care via intravenous fluids. The fevers resolved, but his abdominal pain and jaundice persisted. His t-bili increased without any evidence of infection or renal failure. Hepatology was concerned that he had intrahepatic cholestasis as a result of his SS. They recommended an exchange transfusion and considered a liver transplant. Our patient had many antibodies in his blood, increasing the risk of complications from an exchange transfusion. We deferred a liver biopsy due to the known increased risk of bleeding in SS patients and a lack of impact on management. We continued supportive care, and his liver function improved. Discussion: Acute sickle cell hepatopathy can range from an intra-hepatic sickle cell crisis, acute liver sequestration, and intrahepatic cholestasis; treatments include supportive care and exchange transfusions. Severe cases may require a liver transplant. We were initially concerned for acute intrahepatic cholestasis for our patient, however, his fevers did not persist and he did not have evidence of multi-organ system failure. There was also no evidence of worsening of sickling on a peripheral smear. If his lab abnormalities and symptoms worsened, we would consider doing exchange transfusions or a liver transplant. He improved with conservative measures, and this is an example of a rare complication of a serious disease.