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A Case of Congenital Dyserythropoietic Anemia

Journal of the Korean Pediatric Society 1992;35(4):539-544.
Published online April 15, 1992.
A Case of Congenital Dyserythropoietic Anemia
Il Tae Whang1, Young Sook Ko1, Kyeung Hee Kim1, Gyeung In Lee2, Han Ik Cho3
1Department of Pediatrics, College of Medicine, Ewah Womans University, Seoul, Korea
2Department of Pathology, College of Medicine, Ewah Womans University, Seoul, Korea
3Department of Pathology, College of Medicine, Seoul National University, Seoul, Korea
Congenital Dyserythropoietic Anemia Type I 1례
황일태1, 고영숙1, 김경희1, 이경인2, 조한익3
1이화여자대학교 의과대학 소아과학교실
2이화여자대학교 의과대학 임상병리학교실
3서울대학교 의과대학 임상병리학교실
Abstract
Congenital dyserythropoietic anemia(CDA) is a rare disease characterized by ineffective erythropoiesis, specific cytopathology of the nucleated red cells in the bone marrow and secondary hemochromatosis. CDA is classified into 4 types on the basis of results of electron microscopic and serologic studies. We experienced a case of congenital dyserythropoietic anemia in a 5/12 year-old boy. Diagnosis was established by macrocytosis, anisopoikilocytosis in PB smear and megaloblastoid change in BM findings. A brief review of related literatures was made.
Key Words: Congenital dyserythropoietic anemia


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