Sickle Cell Hepatopathy With Acute Hepatic Sequestration and Extreme Hyperbilirubinemia

Abstract

Sickle cell disease (SCD) is a genetic disorder characterized by chronic hemolytic anemia and multi-organ dysfunction. The liver is frequently affected in SCD, with complications spanning from mild hyperbilirubinemia to acute liver failure. Acute hepatic sequestration (AHS), a rare but serious manifestation of sickle cell hepatopathy, can rapidly progress to multi-organ dysfunction syndrome (MODS). Cases with extreme hyperbilirubinemia (bilirubin > 20 mg/dL) remain exceptionally uncommon. A 19-year-old female patient with SCD presented with nausea, vomiting, generalized pain, dysuria, and suprapubic pain. On presentation, her vital signs were stable except for tachycardia (139 beats per minute (bpm)). Physical examination revealed scleral icterus, conjunctival pallor, and right upper quadrant (RUQ) tenderness. Initial laboratory results showed leukocytosis (white blood cell (WBC): 22.7 bil/L), hemoglobin (Hb) of 7.5 g/dL (baseline: 8.5 g/dL), and platelet (PLT) of 178 bil/L. Lactate dehydrogenase (LDH) was at 734 U/L. Liver function tests showed alkaline phosphatase (ALP) at 238 IU/L, aspartate transaminase (AST) at 52 U/L, alanine transaminase (ALT) at 49 U/L, and total bilirubin at 13.4 mg/dL. Abdominal ultrasound showed hepatomegaly (liver span of 19 cm) and gallstones without signs of cholecystitis. Blood cultures (2/2) tested positive for Escherichia coli (E. coli). Initial management included intravenous (IV) fluids, pain control, IV antibiotics, and two units of packed red blood cells (RBCs). Despite treatment, total bilirubin rose to 35.1 mg/dL with direct bilirubin at >15 mg/dL, hemoglobin dropped to 5.5 g/dL, and platelet count decreased to 124 bil/L. An elevated reticulocyte count (295 × 10⁹/L) and percentage (10.47%) confirmed hepatic sequestration. Exchange transfusion improved the percentage of hemoglobin S (HbS) from 58.9% to 23.4%, decreased bilirubin levels to 12.1 mg/dL, and alleviated symptoms. AHS is rare, occurring in about 1.5% of SCD patients. Extreme hyperbilirubinemia in this case (total bilirubin of 35.1 mg/dL) was more severe than usually seen in AHS, reflecting the severity of the clinical presentation. Differentiating AHS from other hepatic complications, such as acute sickle cell hepatic crisis (ASCHC) and acute intrahepatic cholestasis (AIC), is crucial due to differing management approaches. While ASCHC often responds to supportive care, AHS may require urgent transfusion, and AIC might necessitate invasive interventions, including liver transplantation. Exchange transfusion is effective in AHS by rapidly reducing HbS levels, improving hepatic function, and preventing progression to MODS. Given that AHS is a rare but critical complication of SCD, this case highlights the importance of prompt recognition, a high index of suspicion, and early intervention to optimize outcomes in severe SCD-related hepatic complications.

Keywords: acute hepatic sequestration; acute intrahepatic cholestasis; acute sickle hepatic crisis; double heterozygous hbs and beta thalassemia trait; exchange transfusion; extreme hyperbilirubinemia; hepatic complication of sickle cell disease; sickle cell crisis; vaso-occlusive crisis.