Association between neutropenia and IgG antineutrophil antibodies in a case of CD40LG deficiency due to two novel mutations

Kristian Assing*, Kaspar René Nielsen, Helene Broch Tenstad, Marianne Antonius Jakobsen, Christian Nielsen, Dorthe Grosen, Ulla Birgitte Hartling

*Corresponding author for this work

Research output: Contribution to journalJournal articleResearchpeer-review

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Abstract

This case suggests a mechanistic rationale for the clinical efficacy of intravenous immunoglobulins (IVIG) in treating CD40 ligand (CD40L) deficiency associated neutropenia as it is the first reported instance of free and cell-bound antineutrophil antibodies in a case of CD40L deficiency, accompanied by a prolonged and clinically severe neutropenia.

Original languageEnglish
JournalClinical Case Reports
Volume8
Issue number2
Pages (from-to)313-316
ISSN2050-0904
DOIs
Publication statusPublished - Feb 2020

Keywords

  • CD40 ligand deficiency
  • hyper-IgM syndrome
  • neutropenia
  • neutrophil IgG auto-antibodies

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