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A Case of Congenital Dyserythropoietic anemia Type IV

Journal of the Korean Pediatric Society 1995;38(5):702-706.
Published online May 15, 1995.
A Case of Congenital Dyserythropoietic anemia Type IV
Hwa Young Jeon1, Hwang Min Kim1, Baek Keun Lim1, Duk Woo Park2, Myung Seo Kang2, Young Kun Deung3
1Department of Pediatrics, Wonju College of Medicine, Yonsei University, Wonju, Korea
2Department of Pathology, Wonju College of Medicine, Yonsei University, Wonju, Korea
3Department of Basic Science, Wonju College of Medicine, Yonsei University, Wonju, Korea
Congenital Dyserythropoietic Anemia Type Ⅳ 1례
전화영1, 김황민1, 임백근1, 박덕우2, 강명서2, 등영건3
1연세대학교 원주의과대학 소아과학교실
2연세대학교 원주의과대학 임상병리학과
3연세대학교 원주의과대학 기초과학교실
Abstract
Congenital dyserythropoietic anemia(CDA) is characterized by ineffective erythropoiesis an d increased numbers of multinucleated red cell precursors in the marrow. This syndrome has b een subdassified on the basis of morphologic and serologic differences in the red cell pecursors. There are some patients with congenital dyserythropoietic anemia who do not fit into the conventional classification of type I, II, or III congenital dyserythropoietic anemia. Patients differ from type II congenital dyserythropoietic anemia in that they have a negative reaction in the Ham's test. some investigators consider this a type IV congenital dyserythropoietic anemia. We experienced a case of congenital dyserythropoietic anemia IV in a 3 month-old boy. Diagnosis was established by serologic findings, peripheral smear, bone marrow finding, and electon microscopic findins.
Key Words: Congenital dyserythropoietic anema


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