抗磷脂抗体综合征(APS)课件.ppt

* 参考值：狼疮抗凝物比值（SLC-R）=0.8～1.2。 狼疮抗凝物比值（SLC-R）=狼疮抗凝物质筛选试验检测值（SLC-S）/确诊试验检测值（SLC-C） 狼疮抗凝物（lupus anticoagulant test） * * * APTT（ activated partial thromboplastin time ）： 白陶土、脑磷脂、Ca2＋ →血浆 活化Ⅻ RVVT（ russell viper venom time）： Russel 蟒蛇毒、脑磷脂、Ca2＋ →血浆 活化Ⅹ PT（ prothrombin time ）： 组织因子、磷脂、Ca2＋ →血浆 活化Ⅶ ACT（ activated clotting ttime）： 白陶土→全血 活化Ⅻ RVVT * RVVT（ russell viper venom time） * 不能解释的反复血栓形成 无诱因的大脑或心肌梗塞 少见部位的静脉栓塞 第2、3阶段的妊娠失败 以下情况应立即检查 ------有无APA * ? When testing for aPL is indicated, testing for LA and for IgG antibodies to b2GPI should be performed. The latter can be detected either by an IgG aCL ELISA or an IgGanti-b2GPI ELISA (2C). An aCL ELISA may detect antibodies to other phosphoilipid binding proteins as well as anti-b2GPI. ? In patients with thrombosis, measuring IgM antibodies does not add useful information (2B). ? In patients with pregnancy morbidity, the role of IgM antibodies is unclear (2C). ? Testing for IgA antibodies is not recommended (1B). ? When assessing clinical significance account should be taken of whether the patient has LA, aCL/anti-b2GPI, or both and of the isotype and titre in the solid phase tests (1B). Which tests should be done? LA is the most predictive test for thrombosis and the presence of IgG aCL or IgG anti-b2GPI in those who are Lapositive increases the specificity. There is nothing to suggest that measuring IgM antibodies in patients with thrombosis adds useful information. Tests should be repeated after an interval of 12 weeks to demonstrate persistence. * 体内促凝，体外抗凝？ 1.PT、APTT、RVVT的设计并不是为APS？考察凝血因子 2.如何DD DIC、TTP * * 恶性抗磷脂抗体综合征 (Catastrophic APS) CAPS occurs in w1% of patients with APS but has a 50% mortality rate. 经典APS以大、中血管血栓栓塞为主，多系统受累少见。CAPS以微小血管血栓栓塞为主。CAPS主要临床特点是广泛的血管栓塞事件导致急性多器官功能障碍；受损/坏死组织释放细胞因子等炎性介质导致全身性炎症反应 * * ①溶血性尿毒症综合征(HUS)：起病较急，多见于儿童，在夏季多发，一般与产生志贺