Chronic liver abnormalities in sickle cell disease: a clinicopathological study in 70 living patients.

Background And Aim: The present study aimed to evaluate the incidence and etiology of chronic liver abnormalities in 70 living patients with sickle cell disease from the Hematology and Hemotherapy Center of the State University of Campinas.

Methods: Clinical and laboratory investigations, including liver function tests, serological tests for viral hepatitis and abdominal ultrasound, were performed in all patients. Additionally, liver biopsies were taken from 20 patients.

Results: Sixty-seven (96%) patients had some liver abnormality; these included abnormal liver function tests, viral hepatitis, liver ultrasonographic changes or cholelithiasis. The sickling process was the only explanation for the abnormal liver function tests or liver ultrasonographic changes in 24% of these patients. One or more defined reasons, including viral hepatitis, cholelithiasis, clinical hemosiderosis, alcoholism or diabetes, justified the liver abnormalities in 76% of the patients. Nineteen of the 20 liver biopsies presented some degree of vascular lesion; other histological findings were associated with hemosiderosis, viral hepatitis or cholestasis.

Conclusions: In patients with sickle cell disease, chronic liver abnormalities are frequent and seem to be a multifactorial phenomenon, depending on overlapping factors such as cholelithiasis, viral damage, iron overload and also the primary disease itself.