The most common feature of alpha1-antitrypsin (alpha 1 AT) deficiency is pulmonary emphysema, which becomes manifest in the third to fifth life decades in most subjects with PiZZ phenotype. In recent years, replacement therapy with alpha 1 AT from the plasma of blood donors has been developed. We report the protocol of treatment which has begun to be used in our center, now including two patients. We discuss inclusion criteria, the treatment schedules, the adverse side-effects and the future outlook for this type of therapy.