CAJ | 학술논문

目的探讨继发性噬血细胞性淋巴组织细胞增多症(sHLH)患者血清中新蝶呤(Npt)及腺苷脱氨酶(ADA)的水平及临床意义.方法收集39例初诊sHLH患者血清及其中10例治疗后获得临床缓解患者的血清和15例健康人的血清.采用ELISA方法检测血清Npt水平,用速率法测定血清ADA活性.结果 39例初诊sHLH患者血清Npt水平及ADA活性均显著高于正常对照组[Npt:(164.6±90.0)nmol/L比(7.9±3.6) nmol/L; ADA:(117.2±70.2) U/L比(11.6±4.0) U/L;P值均＜0.001].10例获得临床缓解的sHLH患者治疗后血清Npt水平及ADA活性均较治疗前显著下降[Npt:(17.5±10.9) nmol/L比(170.6±117.9) nmol/L;ADA:(22.5±15.5) U/L比(98.8±52.6)U/L;P值均＜0.05].sHLH患者血清Npt水平与血清可溶性IL-2受体(sCD25)及铁蛋白呈正相关(r =0.526、0.507),血清ADA活性与乳酸脱氢酶呈正相关(r=0.646).血清Npt以148.1nmol/L为最佳临界值时,其判断淋巴瘤相关噬血细胞性淋巴组织细胞增多症(LAHS)的敏感度和特异度分别为70.0％和78.9％;ADA以103.1 U/L为最佳临界值时,其判断LAHS的敏感度和特异度分别为75.0％和84.2％;Npt和ADA联合检测的敏感度为90.0％,特异度为94.7％.结论血清Npt和ADA在sHLH患者的诊断及疗效评估等方面具有重要意义.Npt和ADA可以为疑似LAHS患者提供新的参考指标.
목적 탐토계발성서혈세포성림파조직세포증다증(sHLH)환자혈청중신접령(Npt)급선감탈안매(ADA)적수평급림상의의.방법 수집39례초진sHLH환자혈청급기중10례치료후획득림상완해환자적혈청화15례건강인적혈청.채용ELISA방법검측혈청Npt수평,용속솔법측정혈청ADA활성.결과 39례초진sHLH환자혈청Npt수평급ADA활성균현저고우정상대조조[Npt:(164.6±90.0)nmol/L비(7.9±3.6) nmol/L; ADA:(117.2±70.2) U/L비(11.6±4.0) U/L;P치균＜0.001].10례획득림상완해적sHLH환자치료후혈청Npt수평급ADA활성균교치료전현저하강[Npt:(17.5±10.9) nmol/L비(170.6±117.9) nmol/L;ADA:(22.5±15.5) U/L비(98.8±52.6)U/L;P치균＜0.05].sHLH환자혈청Npt수평여혈청가용성IL-2수체(sCD25)급철단백정정상관(r =0.526、0.507),혈청ADA활성여유산탈경매정정상관(r=0.646).혈청Npt이148.1nmol/L위최가림계치시,기판단림파류상관서혈세포성림파조직세포증다증(LAHS)적민감도화특이도분별위70.0％화78.9％;ADA이103.1 U/L위최가림계치시,기판단LAHS적민감도화특이도분별위75.0％화84.2％;Npt화ADA연합검측적민감도위90.0％,특이도위94.7％.결론 혈청Npt화ADA재sHLH환자적진단급료효평고등방면구유중요의의.Npt화ADA가이위의사LAHS환자제공신적삼고지표.
Objective To investigate the significance of plasma neopterin (Npt) and adenosine deaminase (ADA) in patients with secondary hemophagocytic lymphohistiocytosis (sHLH).Methods Serum specimens from 39 patients with newly diagnosed sHLH,10 sHLH patients who had achieved clinical remission after treatment,and 15 healthy controls were collected.Serum Npt level was detected by enzyme linked immunosorbent assay (ELISA) and ADA activity was tested by kinetic method.Results Npt and ADA values in sHLH group were significantly higher than those in control group [Npt:(164.6 ± 90.0) nmol/L vs (7.9 ± 3.6) nmol/L; ADA:(117.2 ± 70.2) U/L vs (11.6 ± 4.0) U/L; all P ＜ 0.001].Among the 10 sHLH patients who obtained effective clinical treatment,posttreatment levels of Npt and ADA were significantly lower than pretreatment data [Npt:(17.5 ± 10.9) nmol/L vs (170.6 ± 117.9) nmol/L ; ADA:(22.5 ± 15.5) U/L vs (98.8 ± 52.6) U/L; all P ＜ 0.05].The Npt level in sHLH patients was positively correlated with the levels of serum soluble interleukin-2 receptor (sCD25) and serum ferritin (r =0.526 and r =0.507) ; while ADA activity had linear relationship with the level of lactate dehydrogenase (r =0.646).Receiver operating characteristic (ROC) curve analysis showed that 148.1 nmol/L was the critical value of serum Npt for the diagnosis of lymphoma associated hemophagocytic syndrome (LAHS) and the sensitivity and specificity were 70.0％ and 78.9％,respectively.As to ADA,103.1 U/L was the critical value for the diagnosis of LAHS and the sensitivity and specificity were 75.0％ and 84.2％,respectively.The sensitivity and specificity of combined parameters of Npt and ADA were 90.0％ and 94.7％,respectively.Conclusions It is concluded that Npt and ADA have great importance in the diagnosis and evaluation of therapeutic effect in patients with sHLH.Npt and ADA provide potential evidence to diagnose patients who are suspected with LAHS.