GIANT CELL HEPATITIS - TWO CASE REPORTS AND REVIEW OF THE LITERATURE

M Alghamdi, A Bedi, K Peltekian

Dalhousie University, Halifax, Nova Scotia

Giant cell hepatitis with multinucleated syncytial hepatocytes is a common response to injury in the neonatal liver. The adult form, referred to as post infantile giant cell hepatitis (PIGCH), is rare. Multiple etiologies have been cited, with drugs, viruses, and autoimmune mechanisms. We present two variants of this rare disease entity.
The first case was a 49-year-old Caucasian woman diagnosed with pemphigus vulgaris five years ago. Six months later she presented with elevated liver enzymes and non-specific autoimmune markers. Liver biopsy revealed lobular hepatitis and diffuse giant cell transformation with fibrosis. She attained remission with prednisone. Two years ago after stopping prednisone, she presented with decompensated liver disease; biopsy confirmed cirrhosis. She was started on prednisone, could not tolerate azathioprine so was put on low dose cyclosporine. This allowed quick taper of prednisone with normalization of liver enzymes, function and all signs of portal hypertension. She continues to be in remission on cyclosporine.
The second case was a 59-year-old Caucasian woman who presented with acute cholestatic hepatitis, hemolytic anemia and ascites. ANA was positive with high IgG. Liver biopsy revealed fulminant hepatitis with numerous mononuclear cell infiltrates consisting of plasma cells and lymphocytes, together with multinucleated giant hepatocytes. Parvovirus IgM was negative. Despite treatment with intravenous steroids, there was no improvement. She was considered for liver transplantation, but died due to respiratory complications.
Giant cell hepatitis continues to be an uncommon entity in the adult population with limited treatment options.