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- Title
Novel Case of Homozygous Interferon Alpha/Beta Receptor Alpha Chain (IFNAR1) Deficiency With Hemophagocytic Lymphohistiocytosis.
- Authors
Gothe, Florian; Hatton, Catherine F; Truong, Linh; Klimova, Zofia; Kanderova, Veronika; Fejtkova, Martina; Grainger, Angela; Bigley, Venetia; Perthen, Joanna; Mitra, Dipayan; Janda, Ales; Fronkova, Eva; Moravcikova, Dusana; Hambleton, Sophie; Duncan, Christopher J A
- Abstract
We present a case of complete deficiency of the interferon alpha/beta receptor alpha chain (IFNAR1) in a child with fatal systemic hyperinflammation, apparently provoked by live-attenuated viral vaccination. Such pathologic hyperinflammation, fulfilling criteria for hemophagocytic lymphohistiocytosis, is an emerging phenotype accompanying inborn errors of type I interferon immunity.
- Subjects
HEMOPHAGOCYTIC lymphohistiocytosis; IMMUNIZATION; INFLAMMATION; CELL receptors; INTERFERONS; IMMUNOLOGICAL deficiency syndromes; INBORN errors of metabolism
- Publication
Clinical Infectious Diseases, 2022, Vol 74, Issue 1, p136
- ISSN
1058-4838
- Publication type
Article
- DOI
10.1093/cid/ciaa1790