Hemoglobin

Hepatic Crisis in Sickle Cell Anemia

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Hepatic Crisis in Sickle Cell Anemia, Sickle Cell Intrahepatic Cholestasis, Cholelithiasis in Sickle Cell Anemia, Abdominal Pain in Sickle Cell Anemia

  • See Also
  1. Sickle Cell Anemia
  • Epidemiology
  1. Cholelithiasis or biliary sludge Prevalence
    1. Age <18 years: 30 to 50%
    2. Adults: 75 to 85%
  • Pathophysiology
  1. Intrahepatic cholestasis
    1. RBC sickling within liver sinusoids results in hepatic ischemia and Unconjugated Hyperbilirubinemia accumulation
    2. Progressive Intrahepatic cholestasis
      1. Life threatening condition associated with a high mortality rate
      2. Presents with RUQ Abdominal Pain, fever, Vomiting and Jaundice
      3. Risk of Hemorrhage (due to Coagulopathy), encephalopathy and Renal Failure
    3. Contrast with benign cholestasis (benign Hyperbilirubinemia)
      1. Presents only with Jaundice and Pruritus
      2. Common and self-limited, resolving without treatment
  2. Cholelithiasis or biliary sludge
    1. Hemolysis episodes generate higher Red Blood Cell turn-over, resulting in higher Unconjugated Bilirubin
    2. Increased Incidence of Gallstones, sludging, Cholecystitis, Cholelithiasis, Choledocholithiasis, Gallstone Pancreatitis
    3. Asymptomatic Cholelithiasis may be observed as with non-Sickle Cell Anemia patients
    4. Onset in childhood (by age 4 years) and affects up to 75 to 85% of adults
  3. Acute hepatic sequestration
    1. Abnormal Red Blood Cells may be sequestered within the liver
  • Symptoms
  1. Diffuse Abdominal Pain and tenderness
  • Signs
  1. Hepatomegaly (two thirds of SCA patients)
  • Differential Diagnosis
  • Abdominal Pain in Sickle Cell Anemia
  1. Biliary disorders (see above)
    1. Intrahepatic cholestasis
    2. Cholelithiasis
    3. Choledocholithiasis
    4. Ascending Cholangitis
    5. Acute Pancreatitis
  2. Vaso-occlusive crisis
  3. Splenic Sequestration (life threatening)
  4. Intrahepatic sickling (first year of life)
  5. Consider common other intraabdominal conditions
    1. Constipation
    2. Appendicitis
    3. Ovarian Torsion
    4. Ectopic Pregnancy
  • Labs
  1. Elevated Liver Function Tests
  2. Coagulation studies (in Sickle Cell Intrahepatic Cholestasis)
  • Management
  1. Acute Cholecystitis or Choledocholithiasis
    1. Emergent Antibiotics and surgical management
    2. Consult hematology regarding preoperative transfusion
  • References
  1. Dwyer, Kleinmann, Goswami and Lopez (2025) Crit Dec Emerg Med 39(1): 26-35