作者: Richard Hong , William K. Schubert , Eugene V. Perrin , Clark D. West
DOI: 10.1016/S0022-3476(62)80193-0
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摘要: A patient with an uncommon variation of the antibody deficiency syndrome was studied by means quantitation serum immunoglobin response to injected antigens, determination granulocyte kinetics, and skin homografting. The defect defined as a persistent absence beta-2A globulin, severe moderate hypogammaglobulinemia, extreme beta-2 macroglobulinemia (concentrations 400 per cent normal mean). antigens indicated that patient's mechanism for synthesis macroglobulin intact appeared compensate partially failure synthesize nonmacroglobulin antibody. homograft underwent delayed rejection. Survival studies granulocytes showed decreased survival time white cells infused into patient. At age 27 months died alveolar proteinosis Pneumocytis carinii pneumonia. thymus noted be atrophied, peculiar deformed type Hassall's corpuscle found.