Nodular regenerative hyperplasia (NRH) is an uncommon chronic liver disease associated with noncirrhotic portal hypertension. A 29-year-old man with X-linked agammaglobulinemia and NRH complicated by noncirrhotic portal hypertension was followed. Laboratory test results showed pancytopenia and elevated transaminases. Magnetic resonance imaging showed innumerable hepatic lesions. Liver biopsy showed angiosarcoma (hepatic angiosarcoma). He was not a candidate for medical or surgical intervention because of extensive disease and died. Liver histology at autopsy showed infiltrating foci of angiosarcoma. This report extends the literature on a link between malignancy and NRH.
Written work prepared by employees of the Federal Government as part of their official duties is, under the U.S. Copyright Act, a “work of the United States Government” for which copyright protection under Title 17 of the United States Code is not available. As such, copyright does not extend to the contributions of employees of the Federal Government.