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

Farmer, JR; Foldvari, Z; Ujhazi, B; De, Ravin; Chen, K; Bleesing, JJH; Schuetz, C; Al-Herz, W; Abraham, RS; Joshi, AY; Costa-Carvalho, BT; Buchbinder, D; Booth, C; Reiff, A; Ferguson, PJ; Aghamohammadi, A; Abolhassani, H; Puck, JM; Adeli, M; Cancrini, C; Palma, P; Bertaina, A; Locatelli, F; Di, Matteo; Geha, RS; Kanariou, MG; Lycopoulou, L; Tzanoudaki, M; Sleasman, JW; Parikh, S; Pinero, G; Fischer, BM; Dbaibo, G; Unal, EKREM; Patiroglu, TÜRKAN; Karakukcu, MUSA; Al-Saad, KK; Dilley, MA; Pai, SY; Dutmer, CM; Gelfand, EW; Geier, CB; Eibl, MM; Wolf, HM; Henderson, LA; Hazen, MM; Bonfim, C; Wolska-Kuśnierz, B; Butte, MJ; Hernandez, JD; Nicholas, SK; Stepensky, P; Chandrakasan, S; Miano, M; Westermann-Clark, E; Goda, V; Kriván, G; Holland, SM; Fadugba, O; Henrickson, SE; Ozen, A; Karakoc-Aydiner, E; Baris, S; Kiykim, A; Bredius, R; Hoeger, B; Boztug, K; Pashchenko, O; Neven, B; Moshous, D; Villartay, JP; Bousfiha, AA; Hill, HR; Notarangelo, LD; Walter, JE

doi:10.1016/j.jaip.2019.02.038

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

Atıf İçin Kopyala

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

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE, cilt.7, sa.6, ss.1970-1989, 2019 (SCI-Expanded)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 7 Sayı: 6
Basım Tarihi: 2019
Doi Numarası: 10.1016/j.jaip.2019.02.038
Dergi Adı: JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
Sayfa Sayıları: ss.1970-1989
Anahtar Kelimeler: 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
Erciyes Üniversitesi Adresli: Evet

Özet

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.