Autoimmune hepatitis with syncytial giant cells in chronic lymphocytic leukemia: A case report and literature review.

Background: Hepatic manifestations in chronic lymphocytic leukemia (CLL) are common: Elevation of liver enzymes frequently occurs, and differential diagnosis is often challenging. Liver infiltration by leukemic cells, primary and secondary hepatic malignancies, drug-induced hepatotoxicity, immunological disorders, and infections have been reported. Nevertheless, syncytial giant cell hepatitis (GCH) as a manifestation of autoimmune hepatitis in patients with CLL is an extremely rare condition, currently reported only in anecdotal cases.

Case summary: Here, we report the case of a 62-year-old Caucasian woman affected by CLL, who developed GCH with peculiar histopathological features. The patient was evaluated for abnormal liver test results. Liver histology revealed significant inflammatory lymphomononuclear infiltrates with a plasma cell component, widespread syncytial changes in the hepatocytes with gigantocellular features, hepatocyte rosettes, and the typical feature of emperipolesis, consistent with a diagnosis of GCH. The patient was treated with corticosteroids and mycophenolate mofetil, resulting in a complete biochemical response.

Conclusion: Early histological diagnosis of GCH is crucial in patients with CLL, with mycophenolate mofetil representing a promising treatment option.