All issues > Volume 35(11); 1992

Case Report

J Korean Pediatr Soc. 1992;35(11):1578-1583. Published online November 15, 1992.

A Case of Congenital Pelger-Huet Anomaly

Soo Jung SJ Lee¹, Young Yoo YY Kim¹, Seung Won SW Kang¹, Jong Wan JW Kim¹, Kyung Tai KT Whang¹, Sung Ho SH Cho¹

¹Department of Pediatrics, Catholic University Medical College, Seoul, Korea

Abstract

Congenital Pelger-Huet anomaly is an autosomal dominant disorder characterized by incomplete nuclear segmentation of neutrophils without functional disturbance. The morphology of these mature but hyposegmented neutrophils may be confused with that of immature neutrophils (bands), thereby suggesting bacterial infection. We experienced a case of congenital Pelger-Huët anomaly on routine hematologic examination in a 7-year old boy who was admitted because of idiopathic thrombocytopenic purpura and known seizure disorder. And his father had congenital Pelger-Huet anomaly, also. Peripheral blood smear showed that 98% of the neutrophils had unsegmented nuclei, 2% had a bilobed nucleus. Phagocytic and chemotactic activity of patient and his father's neutrophils was normal.

Keywords :Congenital Pelger-Huet anomaly, Phagocytic and chemotactic activity of neutrophils