X-linked agammaglobulinemia (XLA): Phenotype, diagnosis, and therapeutic challenges around the world

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El-Sayed Z. A., Abramova I., Carlos Aldave J., Al-Herz W., Bezrodnik L., Boukari R., ...More

WORLD ALLERGY ORGANIZATION JOURNAL, vol.12, no.3, 2019 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 12 Issue: 3
  • Publication Date: 2019
  • Doi Number: 10.1016/j.waojou.2019.100018
  • Journal Name: WORLD ALLERGY ORGANIZATION JOURNAL
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Keywords: XLA, Agammaglobulinemia, Infection, Autoimmunity, Outcomes, Immunoglobulin, Therapy, PRIMARY IMMUNODEFICIENCY DISEASES, BRUTONS TYROSINE KINASE, PRIMARY HYPOGAMMAGLOBULINEMIA, INTRAVENOUS IMMUNOGLOBULIN, REPLACEMENT THERAPY, CLINICAL-FEATURES, NATIONAL-REGISTRY, BTK, EXPERIENCE, GENE
  • Ankara University Affiliated: Yes

Abstract

Background: X-linked agammaglobulinemia is an inherited immunodeficiency recognized since 1952. In spite of seven decades of experience, there is still a limited understanding of regional differences in presentation and complications. This study was designed by the Primary Immunodeficiencies Committee of the World Allergy Organization to better understand regional needs, challenges and unique patient features.