Leukocyte adhesion deficiency type 1 presenting as leukemoid reaction.

ABSTRACT

Leukocyte Adhesion Deficiency (LAD) is characterized by the inability of leukocytes, in particular neutrophilic granulocytes, to emigrate from the blood stream towards sites of inflammation. Infectious foci are nonpurulent and may eventually become necrotic because of abnormal wound healing. LAD-I is characterized by the absence of the beta-2 integrins CD II and CD 18 on leukocytes. We present a male neonate referred with continuation of leukemoid reaction in spite of antibiotic therapy of simultaneous omphalitis. The diagnosis of LAD-I was confirmed by flow cytometry, showing a deficiency of beta-2 integrins on the surface of the leukocytes. After management, the patient was discharged with prophylactic antibiotics.