From the Research
Hepatic Complications of Sickle Cell Disease (SCD)
The hepatic complications of SCD are diverse and can be acute or chronic. Some of the common hepatic complications include:
- Liver fibrosis and cirrhosis, which can be caused by intravascular hemolysis and vaso-occlusive phenomena, as well as iron overload 1
- Autoimmune hepatitis (AIH), which appears to occur more commonly in the SCD population than in the general population 2
- Sickle cell hepatic crisis, viral hepatitis, sickle cell intrahepatic cholestasis, and hepatic sequestration in the acute setting 2, 3, 4
- Cholelithiasis and iron overload in the chronic setting 1, 2, 5
Pathophysiology and Clinical Presentation
The pathophysiology of hepatic complications in SCD is complex and multifactorial. Intrahepatic sickling of erythrocytes, viral hepatitis, and iron overload secondary to multiple blood transfusions can all contribute to liver disease 5. The clinical presentation of hepatic complications in SCD can range from benign hyperbilirubinemia to overt liver failure 4. Symptoms such as jaundice, fatigue, and abdominal pain are common, as are abnormal liver function tests (LFTs) 2, 3.
Management and Treatment
The management and treatment of hepatic complications in SCD are varied and depend on the specific complication. Exchange blood transfusions may improve outcomes in acute liver syndromes 5, while liver transplantation may be an option for chronic liver disease 5, 4. Corticosteroids and other immunosuppressive agents may be used to treat AIH, but their use in the SCD population can be complicated by the risk of vaso-occlusive crises 2.