alpha 1-Antitrypsin deficiency and hepatocellular carcinoma. Association with cirrhosis, copper storage, and Mallory bodies.
A pathogenetic relationship between hepatic manifestations of alpha 1-antitrypsin deficiency (AATD) and hepatocellular carcinoma has previously been suggested but not established. We encountered three patients with hepatocellular carcinoma (HCC) and nonneoplastic hepatocytes containing globules positive for alpha 1-antitrypsin by immunoperoxidase staining or immunofluorescence microscopy. The AATD was severe in one patient, mild in the second, and not evaluated in the third; any pathogenetic relationship between the deficiency and HCC must therefore remain speculative. Nonneoplastic hepatocytes of all three patients demonstrated copper storage and, in two patients who were not alcoholic, Mallory bodies. Simultaneous presence of copper storage and Mallory bodies has not been reported in liver disease associated with AATD. The pathogenetic mechanisms of these findings are unknown.
D000328:Adult; D000368:Aged; D001706:Biopsy; D006528:Carcinoma, Hepatocellular / Q000150:complications / Q000473:pathology*; D002779:Cholestasis / Q000150:complications; D003300:Copper / Q000032:analysis*; D005260:Female; D005455:Fluorescent Antibody Technique; D006651:Histocytochemistry; D006801:Humans; D007124:Immunoenzyme Techniques; D008099:Liver / Q000473:pathology*; D008103:Liver Cirrhosis / Q000150:complications / Q000473:pathology*; D008113:Liver Neoplasms / Q000150:complications / Q000473:pathology*; D008297:Male; D010641:Phenotype; D019896:alpha 1-Antitrypsin Deficiency
D003300:Copper