Successful allogeneic stem cell transplantation with a reduced-intensity conditioning in a leukocyte adhesion deficiency type I patient.
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.
Hamidieh
Amir Ali
AA
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
Pourpak
Zahra
Z
Alimoghaddam
Kamran
K
Movahedi
Masoud
M
Bahoush
Gholamreza
G
Behmanesh
Fatemeh
F
Moin
Mostafa
M
Ghavamzadeh
Ardeshir
A
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