The Pelger Anomaly of Leukocytes: Forty-One Cases in Seven Families

Abstract

In an investigation of the hereditary nuclear defect of leukocytes originally described by Pelger and by Huet, 7 unrelated instances were identified among 33,492 blood films, an incidence of 1 in 4785. In the course of family studies, 34 additional examples were identified. The defect is characterized by a predominance (69 to 93%) of bilobed mature neutrophils with "pince-nez" nuclei. A few mature neutrophils with round nuclei are also observed. The nuclear chromatin is greatly condensed. The sex chromatin body is not seen in neutrophils of Pelger-positive women. In 4 families, a non-sex-linked dominant inheritance of the Pelger gene was demonstrated. In an extensive study of a fifth family, the anomaly was not found in either maternal or paternal members. A spontaneous mutation is the most likely explanation. There is no apparent relation between the Pelger anomaly and specific illness or predisposition to infection. Leukocytic function is normal.