Alpha 1-antitrypsin deficiency presenting as cryptogenic cirrhosis in adults over 50

We report cirrhosis and alpha 1-antitrypsin deficiency in two patients over 50 years of age who had an initial diagnosis of cryptogenic cirrhosis. Serum alpha 1-antitrypsin levels were in the homozygous range and a liver biopsy demonstrated cirrhosis with periodic acid-Schiff-positive, diastase-resistant globules in both. Red intracytoplasmic inclusion globules seen on trichrome staining in the first patient, and a decreased alpha-1 fraction on serum protein electrophoresis in the second suggested the diagnosis. We propose that the diagnosis of alpha 1-antitrypsin deficiency be considered in all patients, regardless of age, and that trichrome staining be part of the routine histologic evaluation in all patients with cirrhosis of uncertain etiology.