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先天性纯红细胞再生障碍性贫血,亦称Diamond-Blackfan贫血,临床少见,现报道3例如下. 例1:男性,6个月.因反复腹泻,进行性面色苍白4个月,咳嗽、气促5天入院.体检:T38.1℃,R56次/分,P152次/分.发育及营养差,皮肤粘膜苍白,巩膜不黄,鼻翼煽动,哭声低钝、反应迟缓,眼神呆板.心率152次/分,律齐,两肺闻及干湿性罗音.腹平软,肝肋下3cm,脾肋下0.5cm,质软,四肢无畸形.血Hb22.8g/L,WBC6.6×10~9/L,PL225×10~9/L.骨髓象示粒系增生活
Congenital pure red cell aplasia, also known as Diamond-Blackfan anemia, clinical rare, are now reported in 3 cases as follows.Example 1: Male, 6 months due to repeated diarrhea, progressive pale 4 months, cough, shortness of breath 5 days admitted to hospital Physical examination: T38.1 ℃, R56 beats / min, P152 beats / min poor development and nutrition, pale skin mucosa, sclera is not yellow, nose incitement, cry low blunt, slow reaction, eyes dull. Times / min, regular Qi, both lungs and wet and dry rales. Abdomen soft, liver ribs 3cm, 0.5cm spleen ribs, soft, limb deformity. Blood Hb22.8g / L, WBC6.6 × 10 ~ 9 / L, PL225 × 10 ~ 9 / L. Bone marrow imaging showed increased viability