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: Amir Ali Hamidieh, Zahra Pourpak, Kamran Alimoghaddam, Masoud Movahedi, Gholamreza Bahoush, Fatemeh Behmanesh, Mostafa Moin, Ardeshir Ghavamzadeh
• Journal: Pediatric transplantation (Pediatr Transplant) Vol. 15 Issue 2 Pg. E30-3 (Mar 2011) ISSN: 1399-3046 [Electronic] Denmark
• PMID: 19912557 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2009 John Wiley & Sons A/S.

Topics

• Child, Preschool
• Chimerism
• Female
• Follow-Up Studies
• Graft Survival
• Hematopoietic Stem Cell Transplantation (methods)
• Humans
• Leukocyte-Adhesion Deficiency Syndrome (diagnosis, genetics, surgery)
• Risk Assessment
• Transplantation Conditioning (methods)
• Transplantation, Homologous