CLINICAL AND IMMUNOLOGICAL ASPECTS OF HYPER-IGM SYNDROME

ERSOY F., SANAL O., TEZCAN I.

TURKISH JOURNAL OF PEDIATRICS, vol.32, no.1, pp.13-20, 1990 (SCI-Expanded) identifier

  • Publication Type: Article / Article
  • Volume: 32 Issue: 1
  • Publication Date: 1990
  • Journal Name: TURKISH JOURNAL OF PEDIATRICS
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.13-20
  • Hacettepe University Affiliated: No

Abstract

Eight patients with Hyper-IgM syndrome were subjected to clinical and immunological evaluation. There were seven males and one female. All the patients had recurrent pyogenic infections; one had lymphoid hyperplasia with centrally necrotic granulomas, and one had gingivitis with neutropenia. Isohemagglutinin titers were either high or normal in all the patients and five had group O blood. The percentage of IgM-bearing cells were normal in five patients. The percentage of T cells were normal in all the patients, helper T cells were decreased in two patients, and suppressor T cells were increased in four patients. These results suggest that at least in some patients, the imbalances of T cell subsets may play a role in the pathogenesis of the disease rather than it being attributed to an intrinsic B cell defect.