A male or female patient with defective expression of CD18 on leukocytes (less than 5% of normal) and at least one of the following:
A male or female patient with defective expression of CD18 on leukocytes (less than 5% of normal) and all of the following:
Infant with marked leukocytosis (WBC greater than 25,000/mm3) and one of the following:
Marked leukocytosis and recurrent bacterial infections are the hallmarks of LAD. Staphylococcus, gram negative enteric bacteria and fungal infections are particularly troublesome. Periodontitis is a very common persistent finding. In the severe form, no expression of CD18 is detected on neutrophils and early death occurs without BMT. In the moderate form, a small amount of CD18 is expressed and patients can survive to adulthood. Some patients may have normal CD18 expression with a defective CD18 (β2 integrin) activity.