Study of Chronic Hepatopathy in Patients With Sickle Cell Disease

BACKGROUND Hepatic lesions in sickle cell disease were studied essentially in autopsy specimens. We investigated chronic hepatopathy in living adults with sickle cell disease and report the clinical, biochemical, and hepatic histological findings in these patients. METHODS A total of 170 adult patients with sickle cell syndrome were prospectively investigated. Clinical and laboratory investigation including liver function tests, serological tests for viral hepatitis, autoimmune hepatitis, and abdominal ultrasonography were performed in all of the patients. Liver biopsies were studied from 27 patients. RESULTS There was clinical evidence of jaundice in 123 (72.4%) patients, 118 (69.4%) patients had palpable liver, and 69% percent of the patients had elevated enzymes. Serological tests demonstrated the presence of hepatitis B infection in 18 (10.6%) patients and hepatitis C infection in 39 (23%) patients, serological markers for autoimmune hepatitis were positive in two female patients, one diagnosed chronic intrahepatic cholestasis. All 27 biopsies presented some degree of sickling hepatopathy. Moderate or marked liver siderosis was associated with the number of transfusions. CONCLUSIONS The clinical spectrum of sickle cell disease ranges from mild liver function test abnormalities to significant hepatic abnormalities with marked hyperbilirubinemia. Multiple factors may contribute to the etiology of the liver disease, including ischemia, transfusion related viral hepatitis, iron overload, and gallstones.