Variable cell mediated immune defects in a family with 'Candida endocrinopathy syndrome'

S. Kaffe, C. S. Petigrow, L. T. Cahill

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Abstract

The in vivo and in vitro cell mediated immune (CMI) responses of a family of nine members, three of which are affected with 'Candida endocrinopathy syndrome' (CES), have been investigated. No abnormal CMI responses were detected in any of the non affected members. The three patients were found to have low numbers of T cell rosettes. Patient number 1 demonstrated inconsistent in vitro lymphocyte response to Candida extract (CE), with absent migration inhibition factor (MIF) release despite the positive skin reactivity to CE. The patient's serum was found to block the CE induced response of control lymphocytes. Patient number 2 showed absent delayed cutaneous reactivity to CE associated with absent in vitro lymphocyte response, as well as absence of MIF release in response to this antigen. His serum contained inhibitory activity for the Candida induced response of control lymphocytes. Patient number 3 demonstrated similar defects to patient number 1, but the serum blocking factor was not found. Our finding that all affected individuals demonstrated a variety of defects in their CMI responses to CE suggests variable phenotypic expressivity in the presence of the gene responsible for the inheritance of CES. Administration of transfer factor in two of the affected individuals resulted in an increase of T cell rosettes in both patients, conversion of skin reactivity to CE in one of them, and augmentation of skin reactivity to this antigen followed by partial clinical improvement in the other.

Original languageEnglish (US)
Pages (from-to)397-408
Number of pages12
JournalClinical and Experimental Immunology
Volume20
Issue number3
Publication statusPublished - Jan 1 1975
Externally publishedYes

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ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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