Outcomes and Treatment Strategies for Autoimmunity and Hyperinflammation in Patients with RAG Deficiency


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Farmer J., Foldvari Z., Ujhazi B., De R., Chen K., Bleesing J., ...More

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE, vol.7, no.6, pp.1970-1989, 2019 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 7 Issue: 6
  • Publication Date: 2019
  • Doi Number: 10.1016/j.jaip.2019.02.038
  • Title of Journal : JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
  • Page Numbers: pp.1970-1989
  • Keywords: Recombination activating gene (RAG), Severe combined immunodeficiency (SCID), Immune dysregulation, Autoimmune cytopenias, Hematopoietic stem cell transplantation (HSCT), SEVERE COMBINED IMMUNODEFICIENCY, RECOMBINATION ACTIVITY, GRANULOMATOUS-DISEASE, CLINICAL PHENOTYPES, VACCINE-STRAIN, OMENN SYNDROME, MUTATIONS, CYTOPENIAS, RITUXIMAB, GENE

Abstract

BACKGROUND: Although autoimmunity and hyperinflammation secondary to recombination activating gene (RAG) deficiency have been associated with delayed diagnosis and even death, our current understanding is limited primarily to small case series.