重症出凝血疾病详解.ppt

HUS DIC的治疗 DIC治疗的总原则和目的 去除产生DIC的基础疾病及诱因 适当的支持治疗 恢复正常血小板及血浆凝血因子水平 阻断血管内凝血和继发纤溶亢进过程 治疗基础疾病及消除诱因 原发病的治疗是终止DIC病理过程的关键。实践表明，凡是病因能够迅速去除或控制的患者，DIC也易于纠正；相反，基础疾病未去除或难以去除患者，DIC常难以控制、预后不佳。 某些诱因是促发DIC的重要因素，如休克、缺氧、酸中毒、电解质失衡等，积极消除这些诱因有利于DIC的控制。 替代支持治疗 关于替代治疗可能“火上加油（ fuel on the fire ）”的说法并无根据。 替代治疗对于血小板（20?109/L）和Fibrinogen（1.0g/L）过低、有出血的DIC患者十分必要。 如果使用肝素抗凝，也需要在输注替代性治疗前提下进行。 替代支持治疗种类 FFP：使Fib提高到1.0g/L，PT、APTT得到纠正。 血小板：使Plt升至20~30?109/L，如果对患者进行创伤性检查或治疗则需要更多输注。 其它：纤维蛋白原、凝血酶原复合物（PCC）、rhFVIIa. 重建凝血、纤溶的动态平衡 抗凝疗法 肝素 指征：能迅速除去病因的DIC 原则：宜早不宜晚 剂量：遵循个体化原则 无效时考虑：病因未去除、AT- Ⅲ减少 慎用范围：DIC晚期继发纤亢、肝肾功能不全 Heparin has been used historically as a treatment for DIC with various outcomes in different clinical situations, but there is little evidence that heparin reverses organ dysfunction. JMHW approved the LMWH dalteparin for use in DIC ， significantly reduced organ failure and higher safety than the unfractionated version. In patients with high risk of bleeding, unfractionated heparin may be considered due to its shorter half-life and reversibility with protamine sulfate. 肝素在DIC治疗中的作用常常是有限的! 新药应用 1. APC： Unsuccessful 2. AT-III： Unsuccessful 3. TFPI： Unsuccessful 4. Thrombomodulin（血栓调节蛋白 ）:有前景 PROWESS study showed reduced mortality (24.7%) compared with placebo (30.8%) . PROWESS-SHOCK study showed no reduction of mortality, APC was withdrawn from the market. APC AT- Ⅲ Supplemented AT dosing has gained approval in Japan. Treatment for sepsis or DIC is not a licensed indication worldwide. The mortality rates of DIC patients associated with infection was significantly decreased by TM (28.0% vs 34.6%). Significantly lower 28-day mortality，indicating an organ protective effect in the setting of sepsis. TM DIC分期、实验室、治疗总结 Thanks * Scientific and Standardisation Committee of the International Society on Thrombosis and Haemostasis (ISTH/SSC) Classification of DIC types. Coagulation activation (TAT elevation) is a common feature, but the degree of fibrinolytic activation (PIC elevat