Introduction: Intrahepatic vaso-occlusive syndrome, or sickle cell hepatopathy (SCH), is a rare complication of sickle cell disease. We present a case of acute hepatic sequestration in the setting of bacteremia.
Case Description/Methods: 32 male with history of sickle cell disease, auto-splenectomy and end stage renal disease presented with fatigue and diffuse myalgia. Physical exam was notable for scleral icterus, left knee swelling and erythema, and crackles in bilateral lungs. Initial labs showed leukocytosis (WBC 43,000/μL), profound anemia (Hgb 3.7 g/dL) and coagulopathy (INR 2.1). Liver enzymes were elevated at ALP 527 U/L, ALT 48 U/L, AST 209 U/L. Total bilirubin was 39 mg/dL with conjugated fraction 34.4 mg/dL, blood urea nitrogen 67mg/dL, creatinine of 4.34mg/dL, and ferritin >10,000. Blood cultures revealed Staphylococcus Aureus, likely due to left knee septic joint. US showed hepatomegaly with liver span of 24 cm. Clinical picture consistent with sepsis secondary to MSSA bacteremia in setting of sickle cell crisis and hepatopathy. Patient was admitted to ICU for hypoxic respiratory failure due to fluid overload. Clinical course was complicated by septic shock with multi-organ dysfunction including acute liver failure due to hepatic sequestration crisis, hemochromatosis, encephalopathy and disseminated intravascular coagulation. Patient required CRRT and supportive care including treatment of underlying infection, PRBC transfusions, and vitamin K administration to reverse coagulopathy.
Discussion: Hepatopathy occurs in 10% of homozygous sickle cell patients. It can be self resolving or lead to fulminant hepatic failure. Subsets of SCH include Acute Hepatic Crisis(AHC), Hepatic Sequestration, Intrahepatic Cholestasis(IC). Sickling in the sinusoids leading to hepatocellular necrosis, engorgement of Kupffer cells and bile stasis. Common presentation of all three conditions includes acute RUQ pain, nausea, fever, and elevated liver enzymes. IC is severe presentation of SCH with hypoxic injury leading to blockage and cholestasis. Presentation overlaps with AHC with addition of hyperbilirubinemia, severe jaundice, renal impairment, and encephalopathy. Total bilirubin is typically markedly elevated due to combination of hemolysis, cholestasis and renal dysfunction. Sequestration presents with significant rapid onset hepatomegaly, drop in hemoglobin with RUQ pain, which can ultimately lead to cardiac and pulmonary instability. Treatment is supportive with exchange transfusion, fresh plasma for coagulopathy.
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