Nijmegen法检测凝血因子Ⅷ抑制物的临床应用

Detection of factor Ⅷ inhibitor level by the Nijmegen assay and its clinical application

目的观察血友病A(HA)及获得性HA(AHA)患者凝血因子Ⅷ(FⅧ)抑制物的产生情况,探讨Nijmegen法结合APTT纠正试验在FⅧ抑制物检测中的应用及对临床诊断和治疗的指导意义。方法用一期凝固法对42例临床患者进行凝血酶原时间(PT)、活化部分凝血活酶时间(APTT)及FⅧ活性检测,进一步采用APTT纠正试验进行筛选,阳性患者用Nijmegen法进行FⅧ抑制物浓度的测定,并结合临床资料进行分析。结果 42例均为FⅧ活性减低患者。39例HA患者中有2例存在FⅧ抑制物(5.1%),浓度分别为3.7 BU/m L和83.2 BU/m L。3例非HA患者经APTT纠正试验未能纠正到正常范围,其中2例AHA患者的抑制物浓度分别为3 686 BU/m L和61 BU/m L,另1例SLE患者APTT纠正试验结果不具有时间依赖性,抑制物浓度仅为0.1 BU/m L。结论 Nijmegen法结合APTT纠正试验检测FⅧ抑制物,方法简便准确,可为FⅧ活性减低患者的临床诊断和治疗提供指导。

Objective To investigate the development of factor Ⅷ（ FⅧ） inhibitor in the patients with hemophilia A（ HA） or acquired hemophilia A（ AHA）,and evaluate the application of Nijmegen assay combined with activated partial thromboplastin time（ APTT） mixing test in the detection of FⅧ inhibitor and its clinical significance. Methods The prothrombin time（ PT）,APTT and FⅧ activity in 42 patients with reduced FⅧ activity were detected by one-stage clotting assay. Then,the FⅧ inhibitor levels in the patients with positive APTT mixing test were determined by the Nijmegen assay,and the results were analyzed combined with clinical data. Results Two of 39（ 5. 1%） HA patients were detected F Ⅷ inhibitor,and the inhibitor levels were 3. 7 BU / m L and 83. 2BU / m L,respectively. In the other 3 AHA patients,APTT mixing test failed to correct APTT to the normal range. Among them,2 had3 686 BU / m L and 61 BU / m L of FⅧ inhibitors,respectively,and the other with systemic lupus erythematosus（ SLE） only had 0. 1BU / m L of inhibitor and the time-independent result of APTT mixing test. Conclusion The Nijmegen assay combined with APTT mixing test is simple and accurate in the detection of FⅧ inhibitor,which may be helpful in the clinical diagnosis and treatment of the patients with impaired FⅧ activity.