Defective neutrophil mobility in the May-Hegglin anomaly

A case of May-Hegglin anomaly is reported in which functional studies of PMN cells showed abnormalities consisting of impairment of chemotactic and chemokinetic responses, random mobility being otherwise normal. These abnormalities seem unrelated to microtubule system dysfunction or to abnormal cell deformability, since no defect was observed in the concanavalin A (Con A) surface receptors and no improvement of directional movement resulted when filters of larger pore size were used in the assays. Other possible mechanisms of the functional defect, such as abnormal membrane receptors to kinetic signals or metabolic abnormalities cannot be excluded. PMN function should be studied in additional cases in order to ascertain if these findings are a constant feature in the May-Hegglin anomaly, a syndrome in which undue susceptibility to infection has not been reported.