中国实用内科杂志2025，Vol.45Issue(7)：559-563,5.DOI:10.19538/j.nk2025070104
阵发性睡眠性血红蛋白尿症的血管外溶血和突破性溶血
Extravascular hemolysis and breakthrough hemolysis of paroxysmal nocturnal hemoglobinuria
摘要
Abstract
Investigating the mechanisms and management strategies of extravascular hemolysis(EVH)and breakthrough hemolysis(BTH)in patients with paroxysmal nocturnal hemoglobinuria(PNH)following complement inhibitor therapy.EVH is primarily triggered by C3b deposition on erythrocyte surfaces that induces macrophage phagocytosis.Diagnostic criteria include hemoglobin ≤9.5 g/dL or a decrease ≥2 g/dL,accompanied by elevated reticulocytes(≥120×109/L)and C3 deposition on erythrocytes(C3-positive direct Coombs test or C3 fragment detection via flow cytometry).Conventional therapies show limited efficacy,while proximal complement inhibitors(e.g.,Pegcetacoplan,Iptacopan)demonstrate effective control.BTH predominantly results from breakthrough complement activation despite inhibition,diagnosed when LDH exceeds 1.5 times the upper limit of normal with acute hemoglobin decline ≥1.5 g/dL.Severity correlates with complement inhibitor targeting sites and complement-amplifying conditions(CACs).Novel complement inhibitors may optimize PNH hemolysis,though vigilance is required for potential severe BTH following proximal inhibitor therapy.关键词
阵发性睡眠性血红蛋白尿症/血管外溶血/血管内溶血/残存/突破性溶血Key words
paroxysmal nocturnal hemoglobinuria/extravascular hemolysis/intravascular hemolysis/remnant/breakthrough hemolysis分类
医药卫生引用本文复制引用
何广胜,秦程涛..阵发性睡眠性血红蛋白尿症的血管外溶血和突破性溶血[J].中国实用内科杂志,2025,45(7):559-563,5.基金项目
国家自然科学基金(82400138) （82400138）
江苏省自然科学基金(BK20230734) （BK20230734）
