Abstract
A 4½ month old black female with omphalitis at 4 days of age, recurrent infections of the skin, an acquired rectovaginal fistula, marked lymphadenopathy proximal to sites of infection and delayed wound healing had a peripheral WBC count of 70-100,000/mm3 with 70% PMN's and 10% monocytes. PMN's were absent in exudates of infected areas and a Rebuck skin window at 4, 8 and 12 hours. A chemotactic assay under agarose revealed absent directed movement and decreased non-directed movement of PMN's and monocytes. Zymosan activated serum from both patient and normal controls attracted normal cells to the same degree. No serum, plasma or cellular inhibitor to normal PMN movement was found. The patient's mean PMN velocity by direct observation was 2.4 μ/min. (control 11.9 μ/mtn.) and her mean monocyte velocity was 2.1 μ/min. (control 4.5 μ/min.). By light microscopy the patient's cells formed multiple, small lame 11ipodia without developing a single dominant lamel1ipodium; electron microscopy of the cells was normal. Associated findings included marked panhyperimmunoglobulinemia with an IgE of 2600-4300 ng/ml. Assays of the phagocytes' metabolic, phagocytic and bactericidal functions, T and B cell functions and complement system were normal. This black child with severe infections from birth, a markedly elevated IgE without allergic symptoms and defective movement of both PMN's and monocytes represents a congenital and possibly genetically determined abnormality of cell mobility.
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Howard, T., Winkelstein, J., Tsan, MF. et al. CONGENITAL LEUKOCYTE MOVEMENT DISORDER AND RECURRENT INFECTIONS. Pediatr Res 11, 488 (1977). https://doi.org/10.1203/00006450-197704000-00707
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DOI: https://doi.org/10.1203/00006450-197704000-00707