Abstract
Severe alpha 1 -antitrypsin deficiency with phenotype PiZZ was found in association with common variable hypogammaglobulinemia and hyperreactive airways disease in a 34-year-old patient. Immunologic studies demonstrated normal cellular immunity but impaired responses to influenza and pneumococcal antigens. Investigations of the mechanism of hypogammaglobulinemia revealed no intrinsic B cell defect, synthesis abnormality, or impairment of secretion of immunoglobulins. A serum suppressive factor was present and probably accounted for the hypogammaglobulinemia.
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