目的　探讨先天性纯红细胞再生障碍性贫血的早期诊断。方法　观察临床表现、周围血象和骨髓象。结果　贫血发病年龄早 ,生后 10天～ 4个月起病 ,网织红细胞降低 ,白细胞和血小板正常 ,多数患儿骨髓选择性地红系造血障碍 ,部分病儿有遗传性。结论　婴儿早期出现红细胞减少 ,应作网织红细胞计数和骨髓检查 ,以确定诊断 ,避免滥用补血药。 Objective To investigate the early diagnosis of congenital pure red cell aplasia. Methods To observe the clinical manifestations, peripheral blood and bone marrow. Results The age of onset of anemia, onset 10 days to 4 months after onset, reticulocytes decreased, normal white blood cells and platelets, most of the children with selective erythrocyte hematopoietic disorders of bone marrow, some children have hereditary. Conclusion Early erythrocyte reduction occurs in infants. Reticulocyte counts and bone marrow tests should be performed to confirm the diagnosis and avoid abusing the blood drug.