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阵发性睡眠性血红蛋白尿症(ΠΗΓ)属于一种获得性溶血性贫血。有时本症是在再生障碍性贫血的基础上发生的。本病有些患者首先表现为全血细胞减少,仅在作实验室检查时方才发现有中等度的溶血。其余患者溶血以危象形式发生(多在夜间),继以严重贫血。本病的病因学和发病机理至今尚未阐明。按目前的观点,体细胞突变引起红细胞膜的缺陷,从而出现病理性的ΠΗΓ红细胞克隆,这是本病的基础。但ΠΗΓ红细胞缺陷的本质仍不明了。本文应用放射自显影,有核红细胞的多糖组织化学反应,扫描电子显微镜,干扰显微镜和细胞电泳技术。对22例ΠΗΓ患者进行红细胞系列的细胞学比较研究。
Paroxysmal nocturnal hemoglobinuria (ΠΗΓ) belongs to an acquired hemolytic anemias. Sometimes this disease is based on aplastic anemia occurs. Some patients with this disease first showed pancytopenia, only for laboratory tests had just found moderate hemolysis. Hemolysis in the rest of the patients occurred as a crisis (mostly at night) followed by severe anemia. The etiology and pathogenesis of this disease has not yet been elucidated. From the current point of view, somatic mutations cause defects in erythrocyte membranes, resulting in pathological ΠΗΓ red blood cell clones, which is the basis of the disease. However, the nature of ΠΗΓ red cell defects remains unclear. In this paper, autoradiography, polysaccharide histochemical reaction of nucleated erythrocytes, scanning electron microscopy, interference microscopy and cell electrophoresis were used. Twenty-two patients with ΠΗΓ were studied on the cytology of erythrocyte series.