Abstract
Normal family members of eight diagnosed patients suffering from alpha-chain disease had been investigated by immunoelectrophoresis for abnormal alpha-chain protein in their serum. The pattern was demonstrated in four families only, of different members, but all are of the first-degree relationship. The aim of the study was to determine any possible hereditary defect of the immune system in such patients, as compared to the immune system of the normal members of the family who had abnormal protein in their serum. It was found that in both patients and normal members, the proportion of circulating B-lymphocytes was much higher than normal, whereas that of T-lymphocytes was lower than normal. Neither could be sensitized to DNCB, and their skin tests to tuberculin were negative. From all these findings, it was concluded that the disease was a B-cell disease of IgA type transmitted by a hereditary factor associated with a low level of cellular immunity. Further studies are required to support this hypothesis.
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