eISSN: 1644-4124
ISSN: 1426-3912
Central European Journal of Immunology
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1/2020
vol. 45
 
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abstract:
Case report

Successful reconstitution of leukocyte adhesion defect after umbilical cord blood stem cell transplant

Sushmita Chakraborty
1
,
Devika Gupta
1
,
Deepshi Thakral
1
,
Sameer Bakhshi
2
,
Prabin Kumar
1
,
Sushil Kumar Kabra
3
,
Rakesh Lodha
3
,
Dipendra Kumar Mitra
1

  1. Department of Transplant Immunology and Immunogenetics, All India Institute of Medical Sciences, New Delhi, India
  2. Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India
  3. Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India
(Centr Eur J Immunol 2020; 45 (1): 117-121)
DOI: https://doi.org/10.5114/ceji.2020.94713
Online publish date: 2020/04/06
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Leukocyte adhesion deficiencies (LADs) are a type of primary immunodeficiencies characterized by delayed detachment of the umbilical cord, impaired wound healing, leukocytosis, and recurrent infections. The disease is caused by genetic defects affecting different steps in the process of leukocyte adhesion cascade such as rolling, integrin activation, and adhesion of leukocytes, resulting in the impairment of leukocyte trafficking. Till date, three types of LAD have been documented: type I, II and III. Type I LAD is caused by congenital defect in the b2 integrin receptor complex CD11/CD18 on the cell surface of leukocytes, which results in impaired leukocytes connection to endothelial cells and migration. Type II LAD is caused by defect in the fucose metabolism resulting in the absence of fucosylated selectin ligands on neutrophils and impaired rolling phase of the leukocyte adhesion cascade. Type III LAD is caused by mutations in the kindlin-3 gene resulting in defective integrin activation. In this article, we present a review of literature for type I LAD, and successful treatment of patient using umbilical cord blood stem cell transplantation.
keywords:

primary immunodeficiency, leucocyte adhesion defect, immune trafficking, umbilical cord, stem cell transplant
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