Primary Leucocyte Alkaline Phosphatase Deficiency in an Adult with Repeated Infections

An individual with repeated bacterial infections, eczema, hyperimmunoglobulin E, and a primary deficiency of leucocyte alkaline phosphatase is described. The Lap-deficient neutrophils from this patient had marginally deficient bactericidal activity particularly when challenged with high ratios of bacteria per neutrophil. Leucotactic, metabolic and morphologic features of the neutrophils from the patient were normal. Evidence is presented which contrasts this patient's condition with previously described primary or secondary deficiencies of LAP.