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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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

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spelling	doaj-9af74b7fdede4efaa5139548df02efeb2020-11-25T01:10:36ZengWileyClinical Case Reports2050-09042020-02-018231331610.1002/ccr3.2621Association between neutropenia and IgG antineutrophil antibodies in a case of CD40LG deficiency due to two novel mutationsKristian Assing0Kaspar René Nielsen1Helene Broch Tenstad2Marianne Antonius Jakobsen3Christian Nielsen4Dorthe Grosen5Ulla Birgitte Hartling6Department of Clinical Immunology Odense University Hospital Odense DenmarkDepartment of Clinical Immunology Aalborg University Hospital Aalborg DenmarkDepartment of Clinical Immunology Odense University Hospital Odense DenmarkDepartment of Clinical Immunology Odense University Hospital Odense DenmarkDepartment of Clinical Immunology Odense University Hospital Odense DenmarkHans Christian Andersen’s Children Hospital Odense University Hospital Odense DenmarkHans Christian Andersen’s Children Hospital Odense University Hospital Odense DenmarkAbstract 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.https://doi.org/10.1002/ccr3.2621CD40 ligand deficiencyhyper‐IgM syndromeneutropenianeutrophil IgG auto‐antibodies
collection	DOAJ
language	English
format	Article
sources	DOAJ
author	Kristian Assing Kaspar René Nielsen Helene Broch Tenstad Marianne Antonius Jakobsen Christian Nielsen Dorthe Grosen Ulla Birgitte Hartling
spellingShingle	Kristian Assing Kaspar René Nielsen Helene Broch Tenstad Marianne Antonius Jakobsen Christian Nielsen Dorthe Grosen Ulla Birgitte Hartling Association between neutropenia and IgG antineutrophil antibodies in a case of CD40LG deficiency due to two novel mutations Clinical Case Reports CD40 ligand deficiency hyper‐IgM syndrome neutropenia neutrophil IgG auto‐antibodies
author_facet	Kristian Assing Kaspar René Nielsen Helene Broch Tenstad Marianne Antonius Jakobsen Christian Nielsen Dorthe Grosen Ulla Birgitte Hartling
author_sort	Kristian Assing
title	Association between neutropenia and IgG antineutrophil antibodies in a case of CD40LG deficiency due to two novel mutations
title_short	Association between neutropenia and IgG antineutrophil antibodies in a case of CD40LG deficiency due to two novel mutations
title_full	Association between neutropenia and IgG antineutrophil antibodies in a case of CD40LG deficiency due to two novel mutations
title_fullStr	Association between neutropenia and IgG antineutrophil antibodies in a case of CD40LG deficiency due to two novel mutations
title_full_unstemmed	Association between neutropenia and IgG antineutrophil antibodies in a case of CD40LG deficiency due to two novel mutations
title_sort	association between neutropenia and igg antineutrophil antibodies in a case of cd40lg deficiency due to two novel mutations
publisher	Wiley
series	Clinical Case Reports
issn	2050-0904
publishDate	2020-02-01
description	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.
topic	CD40 ligand deficiency hyper‐IgM syndrome neutropenia neutrophil IgG auto‐antibodies
url	https://doi.org/10.1002/ccr3.2621
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Association between neutropenia and IgG antineutrophil antibodies in a case of CD40LG deficiency due to two novel mutations

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