目的:提示患者存在超免疫状态,并表明免疫系统攻击糖基磷脂酰肌醇(GPI)(GPI)细胞,同时逃避GPI细胞免疫。
方法:回顾性分析天津医科大学总医院25例患者外周血免疫细胞亚型,天津,中国,伴有经典阵发性夜间血红蛋白尿(PNH)和50名健康对照。
结果:PNH患者总CD3+和CD3+CD8+细胞水平较高。CD3+细胞呈阳性,与乳酸脱氢酶相关(LDH;r=0.5453,p=0.0040),间接胆红素(r=0.4260,p=0.0379)和单核细胞(r=0.4099,p=0.0303)。然而,CD3+细胞与血红蛋白呈负相关(r=-0.4530,p=0.0105).患者的总CD19+细胞减少,CD19+细胞与单核细胞LDH(r=-0.5640,p=0.0077)和Flear-细胞(r=-0.4432,p=0.0341)呈负相关。患者显示总树突状细胞(DC)的比例增加,在DC群体中具有较高比例的髓样DC(mDC)。此外,红细胞中mDC/DC的比例与CD59-细胞(II+III型)呈正相关(r=0.7941,p=0.0004),粒细胞中的纤维细胞(r=0.5357,p=0.0396),和单核细胞(r=0.6445,p=0.0095)。
结论:我们的结果表明免疫异常与PNH的发生有关。
OBJECTIVE: To suggest the presence of a hyperimmune state in patients, and indicate that immune system attack on glycosylphosphatidylinositol (+) (GPI+) cells while escaping GPI- cell immunity.
METHODS: We retrospective the immune cell subtypes in peripheral blood from 25 patients visiting Tianjin Medical University General Hospital, Tianjin, China, with classical paroxysmal nocturnal hemoglobinuria (PNH) and 50 healthy controls.
RESULTS: The total CD3+ and CD3+CD8+ cell levels were higher in patients with PNH. The CD3+ cells are positively, correlated with lactate dehydrogenase (LDH; r=0.5453, p=0.0040), indirect bilirubin (r=0.4260, p=0.0379) and Flear- cells in monocytes (r=0.4099, p=0.0303). However, a negative correlation was observed between CD3+ cells and hemoglobin (r= -0.4530, p=0.0105). The total CD19+ cells decreased in patients, and CD19+ cells were negatively correlated with LDH (r= -0.5640, p=0.0077) and Flear- cells in monocytes (r= -0.4432, p=0.0341). Patients showed an increased proportion of total dendritic cells (DCs), with a higher proportion of myeloid DCs (mDCs) within the DC population. Moreover, the proportion of mDC/DC was positively correlated with CD59- cells (II + III types) in red cells (r=0.7941, p=0.0004), Flear- cells in granulocytes (r=0.5357, p=0.0396), and monocytes (r=0.6445, p=0.0095).
CONCLUSIONS: Our results demonstrated that immune abnormalities are associated with PNH development.