Open AccessAssociation between neutropenia and IgG antineutrophil antibodies in a case of CD40LG deficiency due to two novel mutations
Author(s) -
Assing Kristian,
Nielsen Kaspar René,
Tenstad Helene Broch,
Jakobsen Marianne Antonius,
Nielsen Christian,
Grosen Dorthe,
Hartling Ulla Birgitte
Publication year - 2020
Publication title -
clinical case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.21
H-Index - 9
ISSN - 2050-0904
DOI - 10.1002/ccr3.2621
Subject(s) - medicine , neutropenia , antibody , immunology , chemotherapy
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.