title: Successful allogeneic stem cell transplantation with a reduced-intensity conditioning in a leukocyte adhesion deficiency type I patient.
abstract: LAD-I is a rare, autosomal recessive, primary immunodeficiency in which phagocyte adhesion and chemotaxis are impaired. Multiple infections in the absence of pus accumulation and persistent elevated peripheral blood neutrophil counts are the hallmark of LAD-I. Allogeneic HSCT is the only treatment proved to be potentially curative for phagocyte adhesion impairment in LAD-I. Here, we report on a case of a 30-month-old girl with LAD-I, in whom peripheral blood stem cell from a genotypically identical sibling resulted in mixed chimerism.
authors.affiliation: Tehran University of Medical Sciences, Hematology-Oncology and Stem Cell Transplantation Research Center, Asthma & Allergy Research Institute, Aliasghar Hospital, Tehran, Iran. aahamidieh@sina.tums.ac.ir
authors.forename: Amir Ali
authors.initials: AA
authors.lastname: Hamidieh
authors.forename: Zahra
authors.initials: Z
authors.lastname: Pourpak
authors.forename: Kamran
authors.initials: K
authors.lastname: Alimoghaddam
authors.forename: Masoud
authors.initials: M
authors.lastname: Movahedi
authors.forename: Gholamreza
authors.initials: G
authors.lastname: Bahoush
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Behmanesh
authors.forename: Mostafa
authors.initials: M
authors.lastname: Moin
authors.forename: Ardeshir
authors.initials: A
authors.lastname: Ghavamzadeh
mesh_terms: D002675:Child, Preschool; D046528:Chimerism; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D018370:Leukocyte-Adhesion Deficiency Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery*; D018570:Risk Assessment; D019172:Transplantation Conditioning / Q000379:methods*; D014184:Transplantation, Homologous