Abstract
A 9-year-old girl was investigated with Job's syndrome to establish the underlying pathogenesis. Recurrent large but “cold” staphylococcal abscesses in skin, kidney and abdomen were unaccompanied by fever, pain and signs of acute inflammation. Normal parameters of the immunological investigation were: cellular, delayed hypersensitivity type of immunity as demonstrated by: 1) skin test response to staphylococcal protein A, SKSD, Candida and challenge with 2,4-DNFB; 2) transformation of lymphocytes with phytohemagglutinin and with staphylococci; 3) skin homograft rejection in 10 days; immunoglobulin levels other than IgE; complement component levels and function; heat stable and labile opsonin activity; in vitro phagocytosis by polymorphonuclear leukocytes of several types of staphylococci including the patient's (P.S.); respiratory enzyme activity of these cells during phagocytosis; in vitro chemotaxis of her leukocytes as generated by P.S. in the Boyden chamber; endogenous pyrogen production by her granulocytes in presence of P.S. The striking abnormalities were: 1) marked scarcity of neu-trophilic granulocytes in staphylococcal abscesses; 2) heavy eosinophilic infiltration of the surrounding tissue, 3) basophilic granulocyte accumulation in inflammatory cycle studies using P.S., and 4) extremely elevated serum IgE level. It is concluded that in vivo chemotaxis for neutrophilic granulocytes is defective in this syndrome.
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Pabst, H., Holmes, B., Quie, P. et al. Immunological abnormalities in Job's syndrome. Pediatr Res 5, 380 (1971). https://doi.org/10.1203/00006450-197108000-00038
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DOI: https://doi.org/10.1203/00006450-197108000-00038
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