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

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

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, a...

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Bibliographic Details
Main Authors: Kristian Assing, Kaspar René Nielsen, Helene Broch Tenstad, Marianne Antonius Jakobsen, Christian Nielsen, Dorthe Grosen, Ulla Birgitte Hartling
Format: Article
Language:English
Published: Wiley 2020-02-01
Series:Clinical Case Reports
Subjects:
CD40 ligand deficiency
hyper‐IgM syndrome
neutropenia
neutrophil IgG auto‐antibodies
Online Access:https://doi.org/10.1002/ccr3.2621
Description
Summary: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.
ISSN:2050-0904

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