摘要
目的探索阵发性睡眠性血红蛋白尿症(PNH)患者血细胞表面补体沉积水平及与临床症状的相关性。方法回顾性收集2021年2月至2023年2月就诊于北京协和医院未经补体抑制剂治疗的PNH患者的临床资料,留取患者外周血样本。选取性别及年龄匹配的正常人作为对照组(NC)。通过流式细胞术检测患者及NC外周血红细胞、白细胞和血小板表面C5b-9、C3、C4b及B因子(FB)的沉积水平,并分析其与患者临床症状的相关性。结果共纳入73例PNH患者,男42例(57.5%),中位年龄36(14~76)岁,NC 16名。PNH患者中,36例(49.3%)为经典型PNH,37例(50.7%)为再生障碍性贫血-PNH综合征;18例(24.7%)曾发生血栓栓塞事件(TEE)。患者中位HGB为76(37~116)g/L,中位网织红细胞绝对计数(Ret)为181.0(45.9~495.8)×10^(9)/L,中位乳酸脱氢酶(LDH)为1875(377~5509)U/L;中位Flaer-白细胞为94.0%(13.0%~99.9%),中位CD59-红细胞为46.7%(9.0%~93.0%)。患者红细胞、白细胞和血小板表面C5b-9、C3、C4b和FB的沉积均显著高于NC(P值均<0.05)。患者上述三类血细胞表面C5b-9的沉积均明显高于C3、C4b和FB(P值均<0.01);所有补体片段在红细胞表面的沉积均低于白细胞和血小板(P值均<0.01)。患者红细胞表面C5b-9的沉积水平与Ret呈正相关(P=0.005);发生TEE者白细胞及血小板表面的C3沉积、白细胞表面的C4b沉积水平均低于未发生TEE者(P值分别为0.001、0.002、0.017)。结论PNH患者血细胞表面C5b-9、C3、C4b和FB的沉积均高于NC,红细胞表面C5b-9水平高可提示活跃溶血,白细胞表面的C3、C4b及血小板表面的C3沉积水平低可提示TEE风险。
ObjectiveTo explore the complement deposition levels on blood cell surfaces in patients with paroxysmal nocturnal hemoglobinuria(PNH)and evaluate their association with clinical manifestations.MethodsThis study enrolled patients with PNH,who had not been treated with complement inhibitors and appeared at Peking Union Medical College Hospital from February 2021 to February 2023.The clinical information of participants was retrospectively recorded,and peripheral blood samples were collected.Gender-and age-matched normal controls(NC)were recruited accordingly.C5b-9,C3,C4b,and factor B(FB)deposition levels on peripheral red blood cells,white blood cells,and platelets were detected with flow cytometry.The correlation between complement deposition levels and clinical symptoms was analyzed.ResultsThis study involved 73 patients with PNH,including 42(57.5%)males,with a median age of 36(range:14-76)years.16 matched NC were collected.Among patients with PNH,36(49.3%)had classical PNH and 37(50.7%)had aplastic anemia-PNH syndrome.Thromboembolic events(TEE)occurred in 18(24.7%)patients.The median HGB,absolute reticulocyte count(Ret),and lactate dehydrogenase of PNH patients were 76(37-116)g/L,181.0(45.9-495.8)×10^(9)/L,and 1875(377-5509)U/L,respectively.The median number of Flaer-negative white blood cells was 94.0%(13.0%-99.9%);the median CD59 negative red blood cells was 46.7%(9.0%-93.0%).The deposition of C5b-9,C3,C4b,and FB on red blood cells,white blood cells,and platelets in patients with PNH was significantly higher than that in NC(all P<0.05).C5b-9 deposition level was significantly higher than that of C3,C4b,and FB on all three blood cell lineages in PNH patients(all P<0.01).The deposition of all complement fragments on red blood cells was significantly lower than that on white blood cells and platelets(all P<0.01).C5b-9 deposition on red blood cells was positively correlated with Ret in PNH patients(P=0.005).C3(P=0.001)and C4b(P=0.017)deposition levels on white blood cells and C3 deposition on platelets(P=0.002)in patients with TEE history were lower than those without.ConclusionsC5b-9,C3,C4b,and FB deposition levels on all three blood cells in patients with PNH were higher than NC.Increased C5b-9 on red blood cells may indicate active hemolysis.Reduced C3 and C4b levels on white blood cells and low C3 deposition on platelets may indicate TEE risk.
作者
张梦露
汪玄
杨辰
陈苗
韩冰
Zhang Menglu;Wang Xuan;Yang Chen;Chen Miao;Han Bing(Department of Hematology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China)
出处
《中华血液学杂志》
北大核心
2025年第4期314-320,共7页
Chinese Journal of Hematology
基金
中国医学科学院临床与转化医学研究专项(CIFMS)(2023-12M-C&T-B-013)
国家自然科学基金(82370121)
北京市自然科学基金(7232109)
中央高水平医院临床科研业务费(2022-PUMCH-C-026、2022-PUMCH-D-002)。
