Leukocyte Adhesion Deficiency: First Case Report from Bangladesh

Abstract

Leukocyte Adhesion Deficiency (LAD) is an autosomal recessive immunodeficiency disorder characterized by failure of leukocyte to adhere to endothelium resulting in recurrent overwhelming infection from childhood. Deficiency of CD18 expression due to mutation of beta-2 integrin results in LAD. Persistent neutrophilia without infection and leukemoid reaction during infection is hallmark for LAD. Based on percentage of CD18 expression it can be divided into three types (mild, moderate and severe). Diagnosis requires flow cytometric measurement of CD 18, CD11a, CD11b and CD15a or mutation analysis. We are presenting a 4-year female baby with recurrent skin and soft tissue infection. Diagnosis was suspected by history, physical examination and blood reports and confirmed by flow cytometric measurement of CD18 expression. It reveals LAD type-I severe variety. Allogenic stem cell transplant is the only recommended option for LAD. In other cases, antibiotic and antifungals are the mainstay of conservative management.