Brain Abscess in a Child with Leukocyte Adhesion Defect: An Unusual Association

Excerpt

Leukocyte adhesion deficiency (LAD) is a primary immunodeficiency caused by a defect in the adherence of neutrophils to the wall of blood vessels. This failure in adherence results in an inability of transmigration of neutrophils through the endothelium to the site of inflammation [1]. Patients with LAD are predisposed to severe and life-threatening bacterial infections from birth. Three types of LAD have been identified thus far [2], namely (i) LAD type I with defect in the ITGB2 gene encoding for CD18, (ii) LAD type II with defect in the SLC35C1 gene encoding for CD15, and (iii) LAD Type III with defect in the FERMT3 gene encoding the Kindlin 3 protein. Patients with LAD fail to develop pus at the site of infection because of an inability of neutrophils and monocytes to egress the circulation and reach the site of inflammation. The most common clinical manifestations include recurrent infections, poor wound healing, ulcers in the oral cavity, and skin abscesses [3]. Delayed separation of the umbilical cord (usually 3 weeks after birth) and omphalitis is a hallmark clinical manifestation of this immunodeficiency albeit not always present. Marked neutrophilic leukocytosis in the peripheral blood is the hallmark laboratory abnormality. Besides, patients with LAD III also have defective platelet aggregation leading to bleeding manifestations and an osteopetrosis phenotype because of a defect in osteoclasts [2]. We report brain abscess in a child with LAD type I, a hitherto unreported clinical presentation in LAD. …