DIC弥散性血管内凝血诊断和治疗进展

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1、DIC的现状 CURRENT ASPECT OF DIC, DIC不是一种独立的疾病而是一个由多种病因引起的出血性病理过程,其特征是微循环内发生广泛的纤维蛋白沉积和血小板聚集,导致弥漫性微血栓形成,继发性凝血因子和血小板大量消耗以及纤溶亢进,从而引起微循环障碍、出血与溶血等一系列严重的临床症状。 国际血栓与止血学会的DIC定义:DIC是多种原因与成分引起的全身性血管内凝血过程。DIC的病理变化主要在微血管,并引起微血管病变,严重时可导致脏器功能障碍。,Underlying Conditions Associated with DICBasic disease ratio of the dise

2、ase to all (%)Infection diseases 36.94 Obstetric complications 24.81 Malignancies 24.21 Surgery and trauma 4.34 Iatrogenic factor 1.45 Other factors 8.25,DIC is characterized by the increasing loss of localization or compensated control in coagulation activation.,DIC pathogenesis is not just related

3、 to “coagulation gone haywire,” but fully involves all components of the inflammatory and innate immune response.,不同原因DIC的临床特征,不同疾病引起的DIC的临床表现不同。败血症DIC易发生肾脏损害;早期以组织缺血为特征,然后才有明显的出血。创伤后DIC可能表现有成人呼吸窘迫综合征。APL引起的DIC主要表现为出血。,弥散性血管内凝血诊断与治疗中国专家共识(2012),临床表现:因原发病不同而差异较大1出血:特点为自发性,严重者可发生危及生命的出血。 2休克或微循环衰竭:早期即

4、出现肾、肺、大脑等器官功能不全。 3微血管栓塞 4微血管病性溶血,弥散性血管内凝血诊断与治疗中国专家共识(2012),DIC的实验室检查包括两方面,一是反映凝血因子消耗的证据,包括(PT、APTT、纤维蛋白原浓度及血小板计数;二是反映纤溶系统活化的证据,包括FDP、D一二聚体、3P试验。,国际血栓与止血学会的分步骤分级诊断标准 1 诱发因素:患者是否有与DIC有关的基础疾病?如果有,继续以下步骤;如果没有,不再继续 2 一般的凝血试验(血小板计数,PT,纤维蛋白原,sFM或FDP) 3 对一般的凝血试验结果进行积分 血小板计数(100 = 0;3sec但6sec = 2) 纤维蛋白原水平(1.

5、0g/l = 0;5:符合DIC;每日重复做检测 如5:提示(但不肯定)为非显性DIC;每12日重复检测,麻省大学医学中心对DIC的常用指标的评价检测指标 敏感性(%) 特异性(%) 诊断效率(%)1 单个试验 血小板计数 97 48 67 PT 91 27 57 APTT 91 42 57 TT 83 60 70 Fbg 22 100 65 AT 91 40 70 FDP 100 67 87 D-D 91 68 80 破碎红细胞 23 73 51 2 联合试验(几个试验均为阳性) PT+APTT+TT 83 11 51 PT+APTT+Fbg 22 100 65 PT+APTT+FDP 91

6、 71 86 FDP+D-D 91 94 95,D-二聚体在DIC患者明显增高,弥散性血管内凝血诊断与治疗中国专家共识(2012),1治疗基础疾病及去除诱因:分别采取控制感染、治疗肿瘤、积极处理病理产科及外伤等措施,是终止DIC病理过程的最为关键和根本的治疗措施。 2抗凝治疗:阻止凝血过度活化、中断DIC病理过程。应在处理基础疾病的前提下,与凝血因子补充同步进行。临床上常用普通肝素和低分子量肝素。,弥散性血管内凝血诊断与治疗中国专家共识(2012),3替代治疗:适用于有明显血小板或凝血因子减少证据且DIC未能得到控制、有明显出血表现者。 (1)新鲜冷冻血浆等血液制品,也可使用冷沉淀。纤维蛋白原

7、水平较低时,可输入纤维蛋白原。,弥散性血管内凝血诊断与治疗中国专家共识(2012),(2)血小板悬液:未出血的患者PLT20109/L,或者存在活动性出血且PLT50109/L的DIC患者。 (3)F及凝血酶原复合物:偶在严重肝病合并DIC时考虑应用。 4其他治疗: (1)支持对症治疗:抗休克治疗,纠正缺氧、酸中毒及水电解质平衡紊乱。,弥散性血管内凝血诊断与治疗中国专家共识(2012),(2)纤溶抑制药物:临床上一般不使用,仅适用于有明显纤溶亢进的临床及实验证据,继发性纤溶亢进已成为迟发性出血主要或唯一原因的患者。 (3)激素治疗:下列情况可予以考虑:基础疾病需糖皮质激素治疗者。感染中毒性休克

8、合并DIC已经有效抗感染治疗者。并发肾上腺皮质功能不全者。,英国DIC治疗指南(2009),The cornerstone of the treatment is treatment of the underlying condition. Transfusion of platelets or plasma should be reserved for patients with bleeding. Severe hypofibrinogenaemia may be treated with fibrinogen or cryoprecipitate. In cases of DIC whe

9、re thrombosis predominates, heparin should be considered. Patients with DIC characterised by a primary hyperfibrinolytic state and who present with severe bleeding could be treated with lysine analogues.,意大利DIC治疗指南(2012),The treatment of the underlying disease. We do not suggest the use of AT or rFV

10、IIa. Heparin or LMWH is not suggested except for thrombo-embombolic prophylaxis in patients without active bleeding. In patients with sepsis/DIC we suggest the use of hr APC. In patients with DIC and active bleeding we suggest transfusion therapy (platelets, plasma, cryoprecipitate). In patients wit

11、h chronic DIC or without active bleeding we do not suggest transfusion therapy based only on laboratory parameters.,Expert consensus for the treatment of DIC in Japan, 2010,In asymptomatic or bleeding DIC, LMWH, synthetic protease inhibitor (SPI), and AT are recommended. In case of severe bleeding,

12、SPI is recommended since it does not cause a worsening of bleeding. Blood transfusions are also required in cases of life threatening bleeding. In the organ failure type, including sepsis, AT has been recommended. DIC with thrombosis and may thus require strong anticoagulant therapy, such as LMWH, U

13、FH, and DS.,DIC and hyperfibrinolysis in acute promyelocytic leukemia,Zhaoyue Wang Jiangsu Institute of Hematology The Affiliated Hospital of Soochow University China,Alterations of SFC, FDP and D-dimer in APL patientsn SFC(mg/L) FDP(pg/L) D-Dimer(pg/L) Control 40 49.716.4 215.363.2 177.143.9 DIC 15

14、 958.6202.3* 764.497.8* 151662788 * Non-DIC 35 316.9195.4* 322.8175.2 23661135 DIC corrected 6 376.7123.6* 366.9113.7 25791679 Compare with control,*P005,*P001, * P 0001;Compare with DIC,P005,P001,P0001,Sepsis-induced DIC with features of TTP: a fatal fulminant syndrome,DIC and TTP are different dis

15、ease states,while ADAMTS13 deficiency could occur in sepsis-induced DIC. We report two patients who had septic DIC with features of idiopathic TTP characterized by low ADAMTS13 activity and positive ADAMTS13 inhibitor. They had a specific fulminant course and fatal outcome, which might represent a new specific syndrome.,

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