Congenital Dyserythropoietic Anemia in Japanese Children

Abstract

Congenital dyserythropoietic anemia (CDA) is a group of conditions characterized by ineffective erythropoiesis and by specific morphologic abnormalities in the bone marrow. Here we report the cases of CDA collected by questionnaire among 220 Japanese hospitals which have pediatric hematology clinics. Twelve cases of pediatric CDA were reported. The male to female ratio was equal. The age of diagnosis ranged from the neonatal period to 4 years. Three cases have family history. The types of CDA were four cases of type I, three cases of type II, two cases of type III and three cases of variant type. Eight cases had the problems during their neonatal period and seven of them were severe jaundice. All but one case received red blood cell transfusion and four of them became transfusion-independent. At the time of this research, seven cases are alive and five cases are dead. The age of death was between eight months and fifteen years old.