本文综合报告先天性纯红细胞再生障碍性贫血20例。男17例,女3例。确诊时17例年龄小于1岁。再生低下性贫血早在出生后发生,但亦可晚于2岁时出现。同胞中具有纯红细胞再生障碍性贫血者2例。苍白、软弱和萎靡常见,仅2例脾大。起病和病程进展缓慢。3例合并先天畸形(先天性心脏病,先天愚型 This article reports 20 cases of congenital pure red cell aplasia. 17 males and 3 females. 17 cases were diagnosed at age less than 1 year old. Reproductive anemia occurs as early as after birth, but may occur later than 2 years of age. Two siblings with pure red cell aplasia in their siblings. Pale, weak and malaise common, only 2 cases of splenomegaly. Slow onset and progression. 3 cases of congenital malformations (congenital heart disease, Down’s syndrome