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β—地中海贫血患者颅内造血国内尚未见报告,现将尸检发现的一例介绍如下。李某,3岁,男性,生后6个月时诊断为重症β—地中海贫血,先后住院15次,共接受输血8300ml。1986年9月16日因肺炎、贫血、心力衰竭入院,20min后死亡。以往住院记载:发育营养欠佳,皮肤粘膜苍白,眼距宽,鼻根塌平,心尖部有Ⅱ级收缩期杂音,腹部膨隆,肝肋弓下10cm,脾肋弓下13cm,中等硬度。RBC1.1×10~(12)/L,WBC21.3×10~9L,Hb18g/L,末梢血片可见到幼稚细胞:原始粒细胞和早幼粒细胞5％,中幼粒细胞7％,
β-thalassemia intracranial hematopoiesis has not been reported in the country, the autopsy found an example described below. Lee, 3 years old, male, diagnosed with severe β-thalassemia 6 months after birth, has 15 hospitalizations, received a total of 8300ml blood transfusion. September 16, 1986 due to pneumonia, anemia, heart failure admitted to hospital, died after 20min. In the past hospital records: poor developmental nutrition, pale skin mucosa, wide eyes, nasal collapse, apical systolic murmur, bulging abdomen, liver ribs under 10cm, spleen ribs under the bow 13cm, moderate hardness. RBC1.1 × 10-12 / L, WBC21.3 × 10-9L, Hb18g / L, peripheral blood cells can be seen naive cells: 5% of the original granulocyte and promyelocytic, 7% of the promyelocytic,