Abstract 878 Hematology-Oncology II Poster Symposium, Sunday, 5/2

Patients with deficiency of PMN function such as leukocyte adhesion deficiency (LAD) exhibit a propensity to bacterial infections and defective wound healing. We present a patient with clinical complications similar to those seen with LAD but normal expression and function of CD11b. The patient had delayed umbilical cord separation at 1 mos. of age and, at 5 wks, developed a perirectal abscess which did not heal in spite of incision, drainage and IV antibiotics. Wound debridement demonstrated tissue necrosis and no pus. At 8 wks, the patient developed a periumbilical abscess and peritonitis. The patient received 8 WBC transfusions over 10 days and IV antibiotics with resolution of his umbilical wound and improvement of the perirectal abscess. Assessment of the patient's PMNs showed a severe motility defect in a modified Boyden chamber; cells failed to migrate into the filter with buffer and exhibited deficient migration (20-25% of control) in response to zymosan activated serum. Bactericidal activity to S. Aureus by patient's PMNs was mildly reduced compared to control. PMNs isolated from the patient and control were evaluated for superoxide anion (O2-) measured as SOD inhibitable cytochrome c reduction, CD11b expression with flow cytometry, and actin assembly with NBD phallicidin in response to a variety of agonists. Shown below are results for O2-. (Table)

Table 1 No caption available.
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