Immunoregulatory abnormalities in mucocutaneous lymph node syndrome

Donald Y M Leung, R. Lawrence Siegel, Stafford Grady, Alan Krensky, Richard Meade, Ellis L. Reinherz, Raif S. Geha

Research output: Contribution to journalArticle

106 Scopus citations

Abstract

The immune status of 21 children in the acute phase of mucocutaneous lymph node syndrome (MCLS) was assessed and compared to that of control populations consisting of age-matched normal children and of children suffering from acute febrile nonbacterial illnesses. In contrast to the controls, 13 of 18 patients studied during the acute phase of MCLS had a significant reduction in circulating T8-positive (T8+) suppressor/cytotoxic T cells (P < 0.001) but normal percentages of T3-positive (T3+) total T cells and of T4-positive (T4+) helper T cells (P > 0.05). Three of three patients were found to have a significantly increased number of Ia-bearing T4+ T cells, suggesting the presence of circulating activated helper cells. Furthermore, 17 of 18 patients with acute MCLS had a significantly elevated number of circulating cells spontaneously secreting IgG (P < 0.01) and IgM (P < 0.001) as determined by a reverse hemolytic plaque assay. Finally mononuclear cells from 18 of 20 patients with acute MCLS had increased cytotoxicity against 51Cr-labeled normal human skin fibroblasts (P < 0.01). Follow-up studies during the convalescence phase of MCLS indicated that most of the patients had gradual resolution of their immunologic abnormalities. These results suggested that immunoregulatory abnormalities may contribute to the pathogenesis of this syndrome.

Original languageEnglish (US)
Pages (from-to)100-112
Number of pages13
JournalClinical Immunology and Immunopathology
Volume23
Issue number1
DOIs
StatePublished - Jan 1 1982

ASJC Scopus subject areas

  • Immunology and Allergy
  • Pathology and Forensic Medicine
  • Immunology

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