Pelger-Huet anomaly in Chinese family in Singapore

Abstract

The Pelger-Huet anomaly consists of failure of segmentation of polymorphonuclear leukocyte nuclei and it is inherited in an autosomal dominant manner. A Chinese family with 5 affected individuals is described and this is the first case described in Singapore. The propositus, in addition, suffered from deafness and chronic renal failure, but both these diseases have no association with the P-H anomaly. The incidence, genetics, mechanism and significance of the anomaly are discussed and is suggested that there may be delayed chemotactic mobility, and this may, in some instances, predispose the patients to repeated infections. The anomaly may be occasionally seen in acquired conditions, especially in granulocytic leukemia and megaloblastic anemia. However, these can be easily distinguished from the genetic variety of P-H anomaly.