血清新蝶呤与腺苷脱氨酶在继发性噬血细胞性淋巴组织细胞增多症患者中的临床意义

The clinical significance of serum neopterin and adenosine deaminase in patients with secondary hemophagocytic lymphohistiocytosis

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.

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.