IMPAIRED NEUTROPHIL CHEMOTAXIS IN PELGER-HUET ANOMALY

  • 1 January 1979
    • journal article
    • research article
    • Vol. 36  (2) , 326-333
Abstract
A family was previously described in which 4 members with Pelger-Huet (P-H) anomaly suffered from recurrent attacks of abdominal pain and fever, while 1 member, whose polymorphonuclear leukocytes (PMN) were also hyposegmented, was asymptomatic. Chemotaxis, chemokinesis and spontaneous locomotion of PMN were studied in the 3 surviving symptomatic sisters, in their asymptomatic brother and 2 asymptomatic members of another family with P-H anomaly. The spontaneous migration of the PMN of the 3 sisters was significantly slower under agarose and in a membrane filter than that of the PMN of the asymptomatic patients with P-H anomaly. Chemotactic and chemokinetic locomotion of the PMN of the symptomatic sisters was also slow. The impaired chemotaxis was apparently due to a defect in the intrinsic locomotor capacity of PMN rather than in their deformability or their responsiveness to the chemotactic stimulus.