The patients studied were diagnosed as suffering from alpha-chain disease by their clinicopathological features, malabsorption findings, X-ray, and presence of abnormal alpha-chain protein in their serum. The objective of the study was to determine any possible defect of the immune system in such patients. The rosette technique and surface immunofluorescence were used to enumerate the circulating T and B lymphocytes in these patients. They were also skin-tested with tuberculin and given sensitizing doses of dinitrochlorobenzene. Their serum immunoglobulins were also quantitated. It was found that the proportion of circulating B lymphocytes was much higher than normal, whereas that of T lymphocytes was lower than normal. Furthermore, they could not be sensitized to DNCB and their skin test to tuberculin was negative. It was concluded that the disease was a B-cell disease of IgA type, associated with low level of cellular immunity.
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