Liver Disease in Hemophilia
Abnormal liver function tests (LFTs) in patients with Hemophilia A and B who have been treated with commercial factor concentrates have been observed since 1973. One hundred and seventeen of these had had LFTs performed at least twice and at least six months apart. Of these, 13 were chronically HbsAg positive and the rest anti-Hbs positive. Forty percent of the anti-Hbs group had persistently abnormal LFTs for greater than 6 months, the majority for more than two years. Owing to the uncertain relationship of this “transaminitis” with true liver pathology, especially in the anti-body positive patients, seven closed liver biopsies were performed without incident under high coverage with factor VIII replacement. All patients were asymptomatic at the time of biopsy. One had a prior episode of illness probably related to subclinical hepatitis. An eighth patient underwent an open liver biopsy as part of a staging laparotomy for Hodgkins Disease. Six biopsies showed varying degrees of chronic persistent hepatitis (CPH). One was not evaluable owing to changes related to a retrospectively obtained history of ETOH intake. Finally, the individual with previous clinical liver disease had post-necrotic cirrhosis. Fluorescent studies with anti-Hbs and-anti-Hbc as well as electron microscopy were done on the seven closed biopsy specimens. The biopsy results suggest that the “transaminitis” found in transfused hemophiliacs represents histologic liver disease. In the majority of patients studied, CPH, a relatively benign entity, was present; and this does not justify withdrawal of concentrate therapy at this time.