先天性纯红细胞再生障碍性贫血6例患儿的临床观察

Congenital pure red blood cell anemia:Clinical study in six infants

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。

Objectives To study the early diagnosis of congenital pure red blood cell anemia.Methods Clinical manifestations,peripheral blood cell count and bone marrow examination.Results The unique features of DBA are.The onset of anemia is early in infancy,always in the first few months after birth;anemia,reticulocytopenia with normal WBC and blood platelet count;the bone manow revealed erythroid hypoplasia or aplasia,the myeloid and megakaryocyte lineages are normal,myeloid and erythroid ratio are increased,several patients had hereditary tendency.Conclusions Erythrocytopenia in early infancy should examine reticulocyte and bone marrow to define the diagnosis,and to avoid abuse hematinics.