All issues > Volume 38(5); 1995

Case Report

J Korean Pediatr Soc. 1995;38(5):702-706. Published online May 15, 1995.

A Case of Congenital Dyserythropoietic anemia Type IV

Hwa Young HY Jeon¹, Hwang Min HM Kim¹, Baek Keun BK Lim¹, Duk Woo DW Park², Myung Seo MS Kang², Young Kun YK 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

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.

Keywords :Congenital dyserythropoietic anema