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

doi:10.1002/ccr3.2621

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

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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 language	English
Journal	Clinical Case Reports
Volume	8
Issue number	2
Pages (from-to)	313-316
Number of pages	4
ISSN	2050-0904
DOIs	https://doi.org/10.1002/ccr3.2621
Publication status	Published - 2020

Bibliographical note

Keywords

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

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10.1002/ccr3.2621Licence: CC BY 4.0

Open Access versionFinal published version, 369 KBLicence: CC BY 4.0

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title = "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, accompanied by a prolonged and clinically severe neutropenia.",

keywords = "CD40 ligand deficiency, hyper-IgM syndrome, neutropenia, neutrophil IgG auto-antibodies",

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AU - Jakobsen, Marianne Antonius

AU - Nielsen, Christian

AU - Grosen, Dorthe

AU - Hartling, Ulla Birgitte

PY - 2020

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AB - 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.

KW - CD40 ligand deficiency

KW - hyper-IgM syndrome

KW - neutropenia

KW - neutrophil IgG auto-antibodies

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

Abstract

Bibliographical note

Keywords

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