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A Case of Congenital Pelger-Huet Anomaly

Journal of the Korean Pediatric Society 1992;35(11):1578-1583.
Published online November 15, 1992.
A Case of Congenital Pelger-Huet Anomaly
Soo Jung Lee, Young Yoo Kim, Seung Won Kang, Jong Wan Kim, Kyung Tai Whang, Sung Ho Cho
Department of Pediatrics, Catholic University Medical College, Seoul, Korea
Congenital Pelger-Huet Anomaly 1례
이수중, 김영유, 강승원, 김종완, 황경태, 조성훈
가톨릭대학교 의과대학 소아과학교실
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.
Key Words: Congenital Pelger-Huet anomaly, Phagocytic and chemotactic activity of neutrophils


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