Leukocyte Adhesion Deficiency Type 1 Presenting as Leukemoid Reaction

The hallmarks of leukocyte adhesion deficiency (LAD) are defects in the leukocyte adhesion process, marked leukocytosis and recurrent infections. These molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of leukocytes from the blood vessels to sites of infection, which requires adhesion of leukocytes to the endothelium. Over last 20 years, three distinctive defects in the leukocyte adhesion cascade, involving several precise ordered steps such as rolling, integrin activation and firm adhesion of the leukocytes have been described. While LAD I and II are clearly autosomal recessive disorders, the mode of inheritance of LAD III is still not clear. LAD I is due to structural defects in the integrin molecule, preventing firm adhesion to occur. We present a case of a male neonate referred with continuation of leukemoid reaction and multiple non healing ulcers, gingivitis and periodontitis in spite of good antibiotic therapy

Keywords:

Leukocyte Adhesion Deficiency Syndrome , Leukemoid Reaction , Neonate