title: Novel adopted immunotherapy for mixed chimerism after unrelated cord blood transplantation in Omenn syndrome.
abstract: Omenn syndrome is a variant form of severe combined immunodeficiency. It is fatal unless treated by allogeneic stem cell transplantation (SCT), which is the only curative approach. However, both treatment-related complications and graft rejection are major obstacles to treatment success. This report describes a case with Omenn syndrome who developed mixed chimerism after unrelated cord blood transplantation (UCBT). This case was successfully treated by altering the patient's immunosuppression and donor lymphocyte infusion (DLI) with donor cord blood-derived activated CD4+ T cells ex vivo expanded from the cord blood cell residues in an infused bag. This novel development to expand CD4+ T-lymphocytes from the donor cord blood unit for the use of DLI would serve as a useful method to overcome the risk of graft rejection in SCT for primary immunodeficiency disorders with residual cell-mediated immunity without compounding graft-vs.-host disease, especially in the UCBT setting.
authors.affiliation: Department of Pediatrics and Developmental Biology, Graduate School, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Tomizawa
authors.forename: Yuki
authors.initials: Y
authors.lastname: Aoki
authors.forename: Masayuki
authors.initials: M
authors.lastname: Nagasawa
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Morio
authors.forename: Michiko
authors.initials: M
authors.lastname: Kajiwara
authors.forename: Teruaki
authors.initials: T
authors.lastname: Sekine
authors.forename: Norio
authors.initials: N
authors.lastname: Shimizu
authors.forename: Masahiko
authors.initials: M
authors.lastname: Kato
authors.forename: Akihiro
authors.initials: A
authors.lastname: Yachie
authors.forename: Shuki
authors.initials: S
authors.lastname: Mizutani
mesh_terms: D015496:CD4-Positive T-Lymphocytes / Q000637:transplantation; D036101:Cord Blood Stem Cell Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007167:Immunotherapy / Q000379:methods*; D007223:Infant; D017710:Lymphocyte Transfusion; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000628:therapy*; D013577:Syndrome; D018183:Transplantation Chimera; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents