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Alpha-1 antitrypsin deficiency has been known to cause pulmonary and hepatic diseases. Cirrhosis in patients with alpha-1 antitrypsin deficiency, especially in a homozygotes ZZ phenotype, has been described to occur exclusively as a congenital disease. We present the case of a young 28-year-old female who was initially followed for thrombocytopenia and was found to have cirrhosis of the liver with autoimmune histological features suggesting the possibility that another “second hit” can contribute to a more rapid progression of liver disease.

Liver Cirrhosis Secondary to Autoimmune Hepatitis in a Patient with Alpha-1 Antitrypsin ZZ Phenotype: A "Double Hit" Phenomenon