PNH者患红细胞膜结构损伤及膜脂与膜蛋白关系的探索

本文分析了20例PNH患者红细胞膜总脂,Ch/PIM比及磷脂组分,并比较了10例PNH红细胞膜AChE在PBS系统和生理盐水中的酶活性。发现PNH红细胞膜脂缺失严重,比正常少22％,但Ch/PIM比值及各磷脂相对含量均无明显改变。在PBS中AChE活性接近正常红细胞,但在生理盐水中明显低于正常红细胞,仅为正常的40％。表明PNH红细胞对补体敏感而溶血可能是由于补体导致膜脂缺失,进而破坏了红细胞膜结构的完整性而引起的,这也是AChE活性在低pH环境中酶活性比正常红细胞低的原因。

Study on Membrane Defects and Relationship between Membrane Lipids and Protein (AChE) of RBC in Patients with Paroxysmal Nocturnal Hemoglobinuria

The content of total lipid, the composition of phospholipid and molar ratio of cholesterol and phospholipid (Ch/P1) of RBC membrane in 20 patients with paroxysmal nocturnal hemoglobinuria (PNH) were analysed, 22% of total lipid of patients' RBC were lost although the composition of pho-spholipid and Ch/P1 did not change significantly. Besides, the activity of AChE of RBC membrane in patients was investigated, and compared with that of control in PBS and normal saline. There was no significant difference in AChE activity between PNH RBC and normal RBC in PBS, but decreased to about 40% of normal value in PNH in normal saline with pH 6. 3. These results suggested that a part of total lipid cf RBC membrane were lost by complement which could cause RBC to be hemolysed with decrease of AChE activity of PNH RBC in normal saline