获得性FⅧ抑制物的实验研究

Experimental studies of Acquired FⅧ Inhibitors

目的 探讨非血友病A中FⅧ抑制物产生的原因及可能机制,并结合其不同的临床表现进行初步研究。方法 采用一期法测定FⅧ:C水平、Bethesda法检测抑制物含量;同时分别用PTT-LA法、ELISA法、APC-Ratio法进行狼疮抗凝物(LA)定性检测、抗心磷脂抗体(aCL)含量测定及活化蛋白C抵抗实验(APCR)。结果 7例病人FⅧ:C水平均明显减低,含量0.4～23.4％,FⅧ抑制物筛选均显示阳性,其抗体滴度从2～15BU不等。结论 获得性FⅧ抑制物的产生大多原因未明,我们的研究提示,一些病例可能与自身免疫有关,如肿瘤,SLE等,获得性FⅧ抑制物的产生可能导致两种临床症状:多数病人表现为不同部位的出血;另一类病人,可能同时出现其它的循环抗体,如抗磷脂抗体,通过多种途径,特别是APCR,而使病人产生高凝状态,导致临床上无明显的出血症状,甚至发生血栓。

Objective To explore the causes and possible mechanisms of F Ⅷ inhibitor in non - hemophilia, with relevant study on a variety of their clinical symptoms. Methods Onestage method and Bethesda assay were used to detect the level of FⅧ: C and its inhibitor. And PPT - LA, ELISA, and APC - ratio methods were used respectively to detect lupus anticoagulants (LA), anticardiolipin antibodies and activated protein C resistance (APCR). Results The level of F Ⅷ: C were significantly reduced in the seven cases, ranging form 0.4% to 23 %. All the screened results of FⅧ inhibitors were positive, and the liters ranged from 2 to 15 BU. Conclusions Most of the causes of acquired FⅧ inhibitor are unknown, but the study indicates that some cases may be relevant to auto - immune diseases such as tumor and SLE. The generation of acquired F Ⅷ inhibitor may induce two clinical symptoms: most cases bleed at different part of the patients and for the others, circulation antibodies such as APA may generate, and by different ways to lead to hyper -coagulation state without clinical bleeding but possible thrombosis.