阵发性睡眠性血红蛋白尿症患者3种GPI锚定蛋白表达的改变

目的 比较阵发性睡眠性血红蛋白尿症(PNH)患者血细胞表面3种糖基化磷脂酰肌醇(GPI)锚定蛋白表达情况。方法 流式细胞术检测25例PNH患者粒细胞表面GPI锚定蛋白CD55、CD59和CD87的表达水平，并分析它们在不同疾病状态下的改变。结果 PNH患者粒细胞表面3种GPI锚定蛋白间具有较好的相关性(P均＜0．05)，红细胞表面CD55和CD59也表现出相关(P＜0．01)，但与粒细胞表面CD55和CD59的缺乏情况并不一致。结论 PNH患者同一系血细胞表面GPI锚定蛋白呈平行关系，这有助于对PNH发病机制的进一步了解。

Alteration of the GPI-Linked Proteins in Patients with Paroxysmal Nocturnal Hemoglobinuria

Objective To investigate the deficient extent of the GPI-linked proteins and their relationship with clinical manifestations in patients with paroxysmal nocturnal hemoglobinuria (PNH). Methods The expression levels of CD55, CD59 on both granulocytes and erythrocytes and CD87 on granulocytes were measured by flow cytometry (FCM) in 25 patients suffered from PNH in different clinical status. Results There were statistically significant coefficients between the expression level of CD55 and that of CD59 on granulocytes ( r =0.66, P <0.01), CD55 and CD87 ( r =0.43, P <0.05), and CD59 and CD87 ( r =0.57, P <0.01). The CD55 and CD59 expression levels on erythrocytes also showed to be correlated. However, they did not always parallel with those on granulocytes in the same patient. Conclusions Our data demonstrated that the GPI-anchored proteins on blood cells in PNH patients were deficient in parallel in the same cell lineage, probably due to a similar mechanism.