title: Acquired Omenn-like syndrome, a novel posttransplant autoaggression syndrome reversed by rapamycin.
abstract: Graft-versus-host disease is uncommon in autologous hematopoietic cell transplantation (HCT) and is typically brief and mild. We report unusual, protracted, and severe Omenn syndrome-like autoaggression following autologous HCT. We identified a profound FOXP3(+) regulatory T cell defect that coincided with hyperinflammatory T cell responses which were reversible with rapamycin in vitro.
authors.affiliation: Division of Infectious Diseases, Department of Medicine, McGill University Health Centre, Montreal, QC, Canada.
authors.forename: Donald C
authors.initials: DC
authors.lastname: Vinh
authors.forename: Khalid
authors.initials: K
authors.lastname: Bin Dhuban
authors.forename: Helen
authors.initials: H
authors.lastname: Mason
authors.forename: Duncan
authors.initials: D
authors.lastname: Lejtenyi
authors.forename: Sungmi
authors.initials: S
authors.lastname: Jung
authors.forename: Donald C
authors.initials: DC
authors.lastname: Sheppard
authors.forename: Damien
authors.initials: D
authors.lastname: Faury
authors.forename: Nada
authors.initials: N
authors.lastname: Jabado
authors.forename: Ciriaco A
authors.initials: CA
authors.lastname: Piccirillo
mesh_terms: D005260:Female; D006086:Graft vs Host Disease / Q000175:diagnosis* / Q000188:drug therapy*; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage*; D008875:Middle Aged; D020123:Sirolimus / Q000008:administration & dosage*; D016176:T-Lymphocyte Subsets / Q000276:immunology; D016896:Treatment Outcome
chemical_list: D007155:Immunologic Factors; D020123:Sirolimus
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