Inherited deficiency of C8 in a patient with recurrent meningococcal infections: Further evidence for a dysfunctional C8 molecule and nonlinkage to the HLA system

P. Densen, E. J. Brown, G. J. O'Neill, F. Tedesco, R. A. Clark, M. M. Frank, D. Webb, J. Myers

Research output: Contribution to journalArticle

12 Scopus citations

Abstract

An adult male with recurrent meningococcal infections is reported whose serum lacked functional C8 activity but possessed antigenic C8. The addition of 1500 U of purified C8/ml of serum restored hemolytic activity to normal. Four to five times more C8 was required to restore bactericidal activity than to restore hemolytic activity. Bactericidal activity could also be restored by mixing the patient's serum with a second C8-deficient serum that lacked detectable antigenic or functional C8. The patient's serum contained bactericidal antibody for groups A, B, C, and Y meningococci and specific antibody to group Y capsular polysaccharide. There was two to three times more bactericidal antibody activity in the serum than in a pool of normal sera for the infecting strain. Family studies disclosed a sibling who was HLA identical to the patient but whose serum contained normal amounts of total hemolytic and C8 functional activity.

Original languageEnglish (US)
Pages (from-to)90-99
Number of pages10
JournalJournal of Clinical Immunology
Volume3
Issue number1
DOIs
StatePublished - Jan 1 1983
Externally publishedYes

Keywords

  • C8 deficiency
  • HLA linkage
  • recurrent meningococcal infection

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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