LAUSR

Leukocyte adhesion deficiency type I (LAD-I) is a rare autosomal recessive primary immunodeficiency disorder that is caused by mutations in ITGB2 gene encoding β2 subunit (CD18) of integrins1. Integrins are required for neutrophils adhesion to endothelium during their emigration to inflammatory site. Hence, integrin deficiency leads to poor neutrophils extravasation, neutrophilia, lack of pus formation and recurrent bacterial infections2. Clinical suspicion is confirmed with integrins detection on neutrophils surface by flow cytometry. Worldwide, only about 300 cases have been reported so far and incidence is one in one million1. However, due to high rate of consanguineous marriages in Pakistan, this incidence is expected to be higher. Previously we had reported a case of LAD-I in 20083, however, since then no new case of the disease has been reported in Pakistan. Here, we present two cases of LAD-I diagnosed in our institute within four months of each other. This article is protected by copyright. All rights reserved.