THE LESSON OF AGAMMAGLOBULINEMIA 40 YEARS AFTER BRUTON DISCOVERY

Forty years after Bruton's discovery, the spectrum of primary defects of immunoglobulins has been largely extended and characterized. An inc reasingly more accurate recognition of the basic pathogenetic mechanis ms of disease has helped to design more effective drugs and therapeuti c strategies for patients with both primary and secondary immune defic iencies. In recent years, major advances in molecular biology have all owed characterization of the genetic basis of many primary immunodefic iencies, resulting in more accurate genetic counseling and leading to the first successful application of genetic therapy to the treatment o f a human disease.