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Journal of Clinical Immunology

, Volume 3, Issue 2, pp 127–134 | Cite as

Immunologic studies of three family members with the immunodeficiency with hyper-IgM syndrome

  • Zacharie Brahmi
  • Kenneth H. Lazarus
  • M. E. Hodes
  • Robert L. Baehner
Original Articles

Abstract

We report the results of immunologic studies in a family in which the father (III-5) and his two daughters (IV-7 and IV-8) had the hyper-IgM syndrome (IHIS). Repeated immunoglobulin levels done on III-5 showed a typical IHIS pattern: low IgG, traces of IgA, and high IgM. IV-7, who also had stage IIA Hodgkin's disease, had a similar pattern except after irradiation therapy to sites of disease, when IgM dropped to normal range while IgG and IgA remained low. IV-8, on the other hand, had normal IgG and IgA and moderately elevated IgM until age 18 months, when she gradually developed the IHIS pattern. All three patients had normal numbers of B cells (sIg) and of T cells, although IV-7 had increased suppression. Finally, all three patients shared the A3,B7 haplotype and none was blood type O. IHIS is not necessarily X linked, is not associated with blood type O, and appears to be heterogeneous even within the same family. Inheritance in this family is apparently autosomal dominant and the father may represent a new mutation.

Key words

Immunodeficiency hyper-IgM Hodgkin's disease cell-mediated immunity 

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Copyright information

© Plenum Publishing Corporation 1983

Authors and Affiliations

  • Zacharie Brahmi
    • 1
  • Kenneth H. Lazarus
    • 1
  • M. E. Hodes
    • 1
  • Robert L. Baehner
    • 1
  1. 1.Departments of Medicine, Microbiology, Pediatric Hematology-Oncology, and Medical GeneticsIndiana University School of MedicineIndianapolis

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