Critical function of the CD40 pathway in parvovirus B19 infection revealed by a hypomorphic CD40 ligand mutation

Frank Blaeser, Michael Kelly, Karen Siegrist, Gregory A. Storch, Richard S. Buller, Jessica Whitlock, Nga Truong, Talal A. Chatila

Research output: Contribution to journalArticle

15 Scopus citations

Abstract

Parvovirus B19-induced chronic anemia has been associated with failure to mount an effective neutralizing antibody response. We describe an adolescent male with a 13-year history of parvovirus B19-induced anemia as the primary manifestation of X-linked hyper IgM immunodeficiency (XHIM). This patient, whose serum IgG concentration was at the low end of the normal range and who mounted IgG antibody responses to T cell-dependent antigens, suffered from a nonsense mutation (R11 → X) in the CD40 ligand (CD40L) gene. This resulted in low-level expression of a mutant CD40L predicted to lack the cytoplasmic domain. Intravenous immunoglobulin therapy alone or in combination with interferon gamma, given in the context of impaired Th1 cytokine production, suppressed but did not eradicate the infection. These results highlight the critical function of the CD40/CD40L pathway in parvovirus B19 infection and suggest that subtle defects in this pathway may underlie cases of chronic parvovirus B19 infection atypical of XHIM.

Original languageEnglish
Pages (from-to)231-237
Number of pages7
JournalClinical Immunology
Volume117
Issue number3
DOIs
StatePublished - Dec 1 2005

Keywords

  • Anemia
  • CD40 ligand
  • Parvovirus B19
  • X-linked hyper IgM immunodeficiency

Fingerprint Dive into the research topics of 'Critical function of the CD40 pathway in parvovirus B19 infection revealed by a hypomorphic CD40 ligand mutation'. Together they form a unique fingerprint.

  • Cite this