Publication:

Leukocyte adhesion deficiency type I in a term neonate with late onset sepsis: a case report

Leukocyte adhesion deficiency (LAD) is a rare primary immunodeficiency disorder of leukocyte function characterized by marked leucocytosis secondary to lack of leukocyte recruitment at the site of infection. LAD type I results from lack of expression of leukocyte cell surface β2 integrins (CD 11 and CD 18) that are essential for leukocyte adhesion to endothelial cells and chemotaxis. LAD I is characterized by delayed separation of the umbilical cord, recurrent life-threatening infections of oral and genital mucosa, skin, intestine and respiratory tract. There is impaired pus formation and delayed wound healing despite extreme neutrophilia. Children with severe form of LAD I (<1% expression of CD18) have the worst prognosis and succumb to infections by 2 years of age. Study reports a case of LAD I in a term neonate who presented with sepsis and the diagnosis was confirmed by flow cytometry. Around 400 cases of LAD I were diagnosed worldwide so far, with very few cases reported from India.