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一、PNH的历史阵发性睡眠性血红蛋白尿症(Paroxysmal Nocturnal Hemoglobinuria,PNH)是一种获得性溶血性疾病,其特点是患者红细胞对血清补体异常敏感。PNH患者不仅其红细胞有异常表现,骨髓造血机能也有不同程度的缺陷。有的病例骨髓造血机能下降到类似于再障贫血的水平。因此现在认为PNH是一种发生于造血干细胞水平的克隆性疾病。PNH在我国北方较多见,西方国家病人为数不多。1867年首次由英国医生William Gull发现。15年以后德国医生PAul Stru-
First, the history of PNH Paroxysmal nocturnal hemoglobinuria (Paroxysmal Nocturnal Hemoglobinuria, PNH) is an acquired hemolytic disease, which is characterized by erythrocyte abnormalities in serum complement abnormalities. PNH patients not only have abnormal performance of red blood cells, bone marrow hematopoietic function also have different degrees of defects. In some cases, bone marrow hematopoietic function is reduced to a level similar to that of aplastic anemia. PNH is now considered a clonal disease that occurs at the level of hematopoietic stem cells. PNH is more common in the north of our country, and there are only a few patients in the western countries. First discovered in 1867 by British doctor William Gull. 15 years later German doctor PAul Stru-