Clinical and Laboratory Factors Affecting the Prognosis of Severe Combined Immunodeficiency

ÖZTÜRK, ELİF; Catak, Mehmet; KIYKIM, AYÇA; Baser, Dilek; Bilgic Eltan, Sevgi; Yalcin, Koray; Kasap, Nurhan; Nain, Ercan; Bulutoglu, Alper; Akgun, Gamze; Can, Yasemin; Sefer, Asena; Babayeva, Royala; Caki-Kilic, Suar; Tezcan Karasu, Gulsun; Yesilipek, Akif; Ozen, Ahmet; Karakoc-Aydiner, Elif; BARIŞ, SAFA

doi:10.1007/s10875-022-01262-0

Clinical and Laboratory Factors Affecting the Prognosis of Severe Combined Immunodeficiency

ÖZTÜRK E., Catak M. C., KIYKIM A., Baser D., Bilgic Eltan S., Yalcin K., ...More

JOURNAL OF CLINICAL IMMUNOLOGY, vol.42, no.5, pp.1036-1050, 2022 (SCI-Expanded, Scopus)

Publication Type: Article / Article
Volume: 42 Issue: 5
Publication Date: 2022
Doi Number: 10.1007/s10875-022-01262-0
Journal Name: JOURNAL OF CLINICAL IMMUNOLOGY
Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, BIOSIS, CAB Abstracts, EMBASE, MEDLINE, Veterinary Science Database
Page Numbers: pp.1036-1050
Keywords: Severe combined immunodeficiency, Bone marrow transplantation, Immune reconstitution, T-cell receptor repertoire, Prognosis, STEM-CELL TRANSPLANTATION, IMMUNE RECONSTITUTION, BONE-MARROW, CHILDREN, SURVIVAL, OUTCOMES
Istanbul University Affiliated: Yes

Abstract

Purpose Severe combined immunodeficiency (SCID) is one of the most severe forms of inborn errors of immunity characterized by absence or loss of function in T cells. The long-term outcomes of all forms of SCID have been evaluated in a limited number of studies. We aimed to evaluate the pre- and post-transplant manifestations of SCID patients and determine the factors affecting the survival of patients.