酸化血清溶血试验阳性的冷凝集素疾病患者临床与实验室特征  

Clinical and Laboratory Characteristics of Cold Agglutinin Disease Patients with Positive Results of Acidified-Serum Lysis Test

作　　者：王朝 王晓雪 安润林 薄丽津 赵玉平 WANG Zhao;WANG Xiao-Xue;AN Run-Lin;BO Li-Jin;ZHAO Yu-Ping(State Key Laboratory of Experimental Hematology,National Clinical Research Center for Blood Diseases,Haihe Laboratory of Cell Ecosystem,Institute of Hematology&Blood Diseases Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Tianjin 300020,China;Tianjin Institutes of Health Science,Tianjin 301600,China)

机构地区：[1]中国医学科学院血液病医院(中国医学科学院血液学研究所),实验血液学国家重点实验室,国家血液系统疾病临床医学研究中心,细胞生态海河实验室,天津300020 [2]天津医学健康研究院,天津301600

出　　处：《中国实验血液学杂志》2025年第2期575-579,共5页Journal of Experimental Hematology

摘　　要：目的:分析酸化血清溶血(Ham)试验阳性冷凝集素疾病(CAD/CAS)的临床与实验室特征,并与Ham试验阴性CAD/CAS及阵发性睡眠性血红蛋白尿症(PNH)比较,以期为相关疾病的鉴别诊断提供参考。方法:回顾性分析2015年1月-2020年12月于本院确诊的CAD/CAS患者53例、经典型PNH患者67例。根据临床诊断及冷凝集素试验(CAT)、直接抗人球蛋白试验(DAT)、Ham试验、PNH克隆检测结果分组,比较各组的临床特征和实验室检查结果。结果:将研究对象分为Ham试验阴性CAD/CAS组(Ham^(-)CAD/CAS组,n=36)、Ham试验阳性CAD/CAS组(Ham^(+)CAD/CAS组,n=17)、经典型PNH组(n=67)。与经典型PNH组相比,Ham^(+)CAD/CAS组的中位年龄较高(P=0.024),Ham试验阳性程度较弱,CAT、DAT阳性率较高,PNH克隆检测阳性率较低(均P<0.001)。Ham^(+)CAD/CAS组脾大、遇冷发绀者比例均明显高于经典型PNH组(P=0.002和P<0.001),红细胞计数(RBC)、乳酸脱氢酶(LDH)均明显低于经典型PNH组(P=0.007和P<0.001),平均红细胞血红蛋白含量(MCH)、平均红细胞血红蛋白浓度(MCHC)、间接胆红素(IBIL)水平均明显高于经典型PNH组(P=0.003、P=0.004和P=0.006)。Ham^(+)CAD/CAS组血清补体C3、C4水平均明显低于经典型PNH组(P=0.001和P<0.001)。Ham^(+)CAD/CAS组尿隐血阳性率明显低于经典型PNH组(P=0.010)。Ham^(+)CAD/CAS组与Ham^(-)CAD/CAS组除中位年龄、血红蛋白(Hb)、MCHC、平均红细胞体积(MCV)、网织红细胞比例(Ret)及Ham试验结果及DAT阳性类型、脾大者比例有明显差别外,其余临床与实验室特征均与Ham^(-)CAD/CAS组接近。结论:Ham试验阳性CAD/CAS患者的部分临床特征及实验室指标与经典型PNH患者存在一定差异,但与Ham试验阴性CAD/CAS患者特征相对接近。这一结果可能为相关疾病的鉴别诊断提供参考。Objective:To analyze the clinical features and laboratory characteristics of patients with cold agglutinin disease(CAD)/cold agglutinin syndrome(CAS)who were positive for acidified-serum lysis test(Ham test),and to compare them with Ham test negative CAD/CAS patients and paroxysmal nocturnal hemoglobinuria(PNH)patients,in order to provide references for the differential diagnosis of these diseases.Methods:53 patients diagnosed with CAD/CAS and 67 patients diagnosed with classic PNH in our hospital from January 2015 to December 2020 were retrospectively analyzed.The patients were grouped according to clinical diagnosis and results of cold agglutinin test(CAT),direct antiglobulin test(DAT),Ham test and PNH clone detection.The clinical and laboratory characteristics of each group were compared.Results:The patients were grouped as follows:Ham^(-)CAD/CAS group,CAD/CAS patients negative for Ham test(n=36);Ham^(+)CAD/CAS group,CAD/CAS patients positive for Ham test(n=17);classic PNH group(n=67).Compared with the classic PNH group,the Ham^(+)CAD/CAS group had a higher median age(P=0.024),weaker positivity of Ham test,higher positive rates of CAT and DAT,and lower positive rate of PNH clone detection(all P<0.001).The proportions of patients with splenomegaly and cyanosis in Ham^(+)CAD/CAS group were significantly higher than those in classic PNH group(P=0.002 and P<0.001).Ham^(+)CAD/CAS group displayed lower red blood cell count(RBC)and lactate dehydrogenase(LDH)level(P=0.007 and P<0.001),and higher mean corpuscular hemoglobin(MCH),mean corpuscular hemoglobin concentration(MCHC),and indirect bilirubin(IBIL)level(P=0.003,P=0.004 and P=0.006)than those in classic PNH group.The levels of serum complement C3 and C4 in Ham^(+)CAD/CAS group were lower than those in classic PNH group(P=0.001 and P<0.001).The positive rate of urinary occult blood in Ham^(+)CAD/CAS group was lower than that in classic PNH group(P=0.010).The clinical and laboratory characteristics of Ham^(+)CAD/CAS group were similar to those of Ham^(-)CAD/CAS grou

关 键 词：酸化血清溶血试验 冷凝集素病/冷凝集素综合征 临床特征 实验室特征 

分 类 号：R556.6[医药卫生—血液循环系统疾病]