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报告两例特殊红细胞形态的先天性溶血性贫血。红细胞形态在血涂片上极似球形红细胞症,但红细胞渗透脆性正常,扫描电镜未见球形红细胞,却见一种特殊形态的红细胞。实验室检查两例患者都附合溶血性贫血特征,但排除酶缺陷、血红蛋白病变、自身免疫性溶血性贫血和阵发性睡眠性血红蛋白尿症等症。扫描电镜下两例红细胞都显示一种特殊形态:红细胞一面出现较大的芽孢状突起,多为一个,往往位于一面的中央。此种红细胞在瑞氏染色的血涂片上不易被发现,但仔细观察可见到与此种红细胞相关的一些形态变化。作者认为该二例是一种特殊类型的红细胞膜形态异常先天性溶血性贫血。由于血涂片特征极似球形红细胞症,临床上尤需与该症作鉴别。
Two cases of congenital hemolytic anemia of special red blood cell morphology were reported. Erythrocyte morphology in the blood smear very similar to spherocytosis, but the normal infiltration of erythrocyte fragility, scanning electron microscopy, no spherical red blood cells, but found a special form of red blood cells. Laboratory tests Both patients had hemolytic anemia features, but excluded enzyme deficiencies, hemoglobinopathy, autoimmune hemolytic anemia and paroxysmal nocturnal hemoglobinuria embolism. Scanning electron microscopy of two cases of red blood cells have shown a special form: red blood cells appear larger spores on one side, mostly one, often located in one side of the center. Such red blood cells in Wright’s stain blood smear is not easy to be found, but a closer look can be seen with some of the red blood cell-related morphological changes. The authors believe that the two cases is a special type of erythrocyte membrane abnormalities congenital hemolytic anemia. Due to the characteristics of blood smear like spherical polycythemia, clinically with the disease especially for identification.