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

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, accompanie...

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Detalles Bibliográficos
Autores principales: Assing, Kristian, Nielsen, Kaspar René, Tenstad, Helene Broch, Jakobsen, Marianne Antonius, Nielsen, Christian, Grosen, Dorthe, Hartling, Ulla Birgitte
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2019
Materias:
Case Reports
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7044354/
https://www.ncbi.nlm.nih.gov/pubmed/32128179
http://dx.doi.org/10.1002/ccr3.2621
Descripción
Sumario: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.

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