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目的 探讨血清乳酸脱氢酶（LDH）水平与阵发性睡眠血红蛋白尿（PNH）患者临床特点和预后的相关性。方法 选取2018年1月至2023年8月收治的PNH患者82例作为研究对象，收集所有患者的临床资料、LDH/正常上限（ULN），根据血清LDH/ULN水平进行分组，血清LDH/ULN≥1.5患者作为溶血组，血清LDH/ULN<1.5患者作为非溶血组，比较两组临床特点、实验室检查指标[血红蛋白（Hb）、白细胞计数（WBC）、血小板计数（PLT）、网织红细胞绝对值（Ret）、粒细胞PNH克隆比例、红细胞PNH克隆比例]、LDH/ULN，分析血清LDH/ULN与临床特点的相关性，并随访1年，统计所有患者的预后情况，对比不同预后患者血清LDH/ULN水平、临床特点，分析血清LDH/ULN与预后的关系，评估血清LDH/ULN对PNH预后的预测价值。结果 溶血组腹痛、吞咽困难、呼吸困难、血栓栓塞（TE）事件比例、血清LDH/ULN、粒细胞PNH克隆比例、红细胞PNH克隆比例高于非溶血组，Hb、WBC、PLT、Ret低于非溶血组（P<0.05,P<0.01）；血清LDH/ULN与腹痛、吞咽困难、呼吸困难、疲劳、TE事件、粒细胞PNH克隆比例、红细胞PNH克隆比例呈正相关，与Hb、WBC、PLT、Ret呈负相关（P<0.01）；预后不良组腹痛、吞咽困难、呼吸困难、疲劳、TE事件、骨髓功能衰竭（BMF）比例、血清LDH/ULN、粒细胞PNH克隆比例、红细胞PNH克隆比例高于预后良好组，Hb、WBC、PLT、Ret低于预后良好组（P<0.05,P<0.01）；腹痛、吞咽困难、呼吸困难、TE事件、BMF、血清LDH/ULN是PNH患者预后不良的独立危险因素（P<0.01）；血清LDH/ULN、Hb、WBC、PLT、Ret、粒细胞PNH克隆比例、红细胞PNH克隆比例预测PNH患者预后的曲线下面积（AUC）分别为0.847、0.734、0.706、0.730、0.729、0.723、0.712，其中血清LDH/ULN单独预测PNH患者预后的AUC最大（P<0.05）。结论 血清LDH/ULN与PNH患者临床特点、预后有关，其中腹痛、吞咽困难、呼吸困难、TE事件、BMF、血清LDH/ULN是PNH患者预后不良的独立危险因素。血清LDH/ULN可用于评估PNH患者预后。
Abstract：Objective To investigate the correlation of serum lactate dehydrogenase(LDH) level with clinical characteristics and prognosis in patients with paroxysmal nocturnal hemoglobinuria(PNH). Methods A total of 82 PNH patients admitted to our hospital from January 2018 to August 2023 were selected as the research subjects. The clinical data and LDH/upper limit of normal(ULN) ratio of all patients were collected. The patients were grouped according to the serum LDH/ULN level, with those having serum LDH/ULN ≥ 1.5 as hemolysis group, and those having serum LDH/ULN<1.5 as the non-hemolysis group. The clinical characteristics, laboratory examination indexes [hemoglobin(Hb), white blood cell count(WBC),platelet count(PLT), reticulocyte absolute value(Ret), PNH clone proportion of granulocytes, PNH clone proportion of erythrocytes] and LDH/ULN were compared between the two groups. The correlation between serum LDH/ULN and clinical characteristics was analyzed, and the prognosis of all patients was recorded after 1 year of follow-up. The serum LDH/ULN levels and clinical characteristics of patients with different prognosis were compared, and the relationship between serum LDH/ULN and prognosis and the predictive value of serum LDH/ULN for prognosis of PNH were analyzed. Results The proportions of abdominal pain, dysphagia, dyspnea, thromboembolism(TE) events, serum LDH/ULN, PNH clone proportion of granulocytes and PNH clone proportion of erythrocytes in hemolysis group were higher than those in non-hemolysis group, while Hb, WBC, PLT and Ret were lower than those in non-hemolysis group(P<0.05, P<0.01). Serum LDH/ULN was positively correlated with abdominal pain,dysphagia, dyspnea, TE events, the PNH clone proportion of granulocytes and the PNH clone proportion of erythrocytes, and negatively correlated with Hb, WBC, PLT and Ret(P<0.01). Compared with the good prognosis group, the poor prognosis group had significantly higher rates of abdominal pain, dysphagia, dyspnea, fatigue, TE events, bone marrow failure(BMF), serum LDH/ULN, the PNH clone proportion of granulocytes and the PNH clone proportion of erythrocytes, and significantly lower Hb, WBC, PLT, and Ret(P<0.05, P<0.01). Abdominal pain, dysphagia, dyspnea, TE events, BMF, and serum LDH/ULN were independent risk factors for poor prognosis in PNH patients(P<0.01). The areas under the ROC curve(AUC) of serum LDH/ULN, Hb, WBC, PLT, Ret, PNH clone proportion of granulocytes, and PNH clone proportion of erythrocytes for predicting the prognosis of PNH patients were 0.847, 0.734,0.706, 0.730, 0.729, 0.723, and 0.712, respectively. Among them, serum LDH/ULN ratio alone had the largest AUC for predicting the prognosis of PNH patients(P<0.05). Conclusion The serum LDH/ULN ratio is related to the clinical characteristics and prognosis of PNH patients. Abdominal pain, dysphagia, dyspnea, TE events, BMF and serum LDH/ULN ratio are independent risk factors for poor prognosis of PNH patients. Serum LDH/ULN ratio can be used to evaluate the prognosis of PNH patients.
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基本信息:
DOI：
中图分类号:R556.64
引用信息:
[1]于艳芳,王九凤,申娜等.血清乳酸脱氢酶水平与阵发性睡眠血红蛋白尿患者临床特点和预后的相关性[J].临床误诊误治,2025,38(12):32-37.
基金信息:
河北省重点研发计划项目（21377797D）; 邯郸市科学技术局项目（23422083363）