彌散性血管內(nèi)凝血DIC.ppt

彌散性血管內(nèi)凝血 Chapter 11,中山醫(yī)學(xué)院病理生理教研室 鄧宇斌,DIC 一、DIC原因和發(fā)病機制 二、促進DIC發(fā)生發(fā)展的因素（誘發(fā)困素） 三、DIC的分期和分型 四、DIC的功能代謝變化（病理生理變化） 五、DIC防治的病理生理基礎(chǔ),第一節(jié) 概述,1.血液的凝固與抗凝 流動性 血液運輸載體 方向性 內(nèi)（） 凝血系統(tǒng) 凝血 外（） 血小板 ： 粘 聚 釋,凝抗凝 栓塞 失衡 凝抗凝 出血傾向 2.DIC的概念 出血 病因 微血栓 后 休克 致凝 繼發(fā)纖溶亢進 果 栓塞 溶血 120種?。焊腥尽⒛[瘤、產(chǎn)科意外,Introduction,DIC is characterized by the activation of the coagulation system with resultant consumption of a variety of coagulation proteins and platelets, which results in hemorrhagic diathesis and ischemic injury to various tissues.,1.Blood Coagulation It is propagated by an enzymatic events termed coagulation cascade. Contact factors and the intrinsic pathway Tissue factor and extrinsic pathway,2.Fibrinolysis It is the result of the action of plasmin, a proteolytic enzyme produced from an inert plasma precursor (plasminogen) by the action of various substances termed plasminogen activators.,Humoral plasminogen activators Tissue plasminogen activators Fibrin or fibrinogen degradation products FDP (Significant biological activity) Fragments X, Y and E (potent antithrombins) Fragments Y and D ( inhibit fibrin polymerization),a a a PC APC TM+ 滅 活 PS(+) C4b C4b PS(-),酶 纖溶 FDP 酶 AT PC 抗 APC TM+a 凝 PS PGI2 VEC TM,第二節(jié) DIC的病因發(fā)病學(xué),一、發(fā)病原因及機理 1.VEC廣泛受損 原因 感染 炎癥、免疫損傷（抗磷脂綜合征） 高低溫、放射損傷 缺血缺氧 酸中毒,Etiology of DIC,1.acute DIC (1)septicemia (2)severe trauma (3)obstetric accidents (4)shock 2.subacute DIC (1)malignant tumors (2)retained dead fetus 3.chronic DIC (1)giant hemangioma (2)systemic lupus erythematosus (SLE),機理 膠原暴露 凝 VEC 釋放 受損 合成PGI2TXA2 抗凝 表達(dá)TM APC,2.血細(xì)胞大量受損 RBC受損 感染：瘧疾 原因：溶血 G6PDase：蠶豆病 免疫損傷：異型輸血 紅細(xì)胞素（） 機理： 釋 ADP P聚集,WBC激活或受損 壞死 白血病細(xì)胞 釋 原因 化療受損 機理 炎癥激活 合成 、釋 （內(nèi)毒素、補體、LC、P、AgAb）,P激活或受損 原發(fā)性：免疫損傷（抗P抗體抗磷脂抗體） 繼發(fā)性：DIC 粘（GPb膠原） 聚（GPba fg） TXA2等 P聚、血管收縮 機理 PF111 提供“反應(yīng)面” aCa2+a aCa2+a PF3 PF3,3.大量致凝物質(zhì)入血 腫瘤細(xì)胞 壞死（包括產(chǎn)科意外） 組織細(xì)胞 帶負(fù)電顆粒物質(zhì)（內(nèi)毒素）a 胰蛋白酶 其它絲氨酸蛋白水解酶 a 蝰蛇毒,Pathogenesis of DIC,1.extensive damage of vascular endothelial cells Intrinsic clotting cascade 2.severe tissue injury Extrinsic clotting reaction,3.excessive destruction of the circulating blood cells Generation of procoagulant-active substances Intravascular coagulation 4.other thromboplastic materials entering the blood Activation of clotting system through the contact of blood with an abnormal surface,the net effects are summarized as follows: 1.loss of plasma fibrinogen as it is consumed by the clotting process and by the action of plasma. 2.loss of other clotting factors notably , and , as they are used up during the operation of the clotting cascade. 3.fall in the platelet count, as the platelets aggregate and leave the circulation. 4.appearance of fibrin degradation products, as plasmin acts on its substrates.,二、誘因與發(fā)生機理 消除致凝物質(zhì)功能 血液凝血活性抗凝活性 1.單核吞噬細(xì)胞系統(tǒng)功能 內(nèi)毒素血癥、糖皮質(zhì)激素、脾 消除功能 ：致凝物、a 、凝纖產(chǎn)物,2.肝功能嚴(yán)重障礙 滅活活化凝血因子 合成AT、PC 枯否細(xì)胞吞噬功能 3.血液的高凝狀態(tài) 凝血活性 凝血物質(zhì)：懷孕、腫瘤、應(yīng)激 抗纖溶：胎盤、藥 抗凝活性 抗肝素：H AT、PC、TM等 4.血流郁滯,Predisposing factors to DIC,1.impairment of the clearance mechanism. 2.hypercoagulable state. 3.disorder of microcirculation.,第三節(jié) DIC的分期及分型,高凝期 分期 消耗性低凝期 繼發(fā)性纖溶亢進期 急性 按發(fā)病速度 亞急性 分 慢性 型 代償型 按代償情況 失代償型 過度代償型,Types of DIC 1.acute DIC Multiside bleeding diathesis Thrombotic complications usually Severe bleeding lead to shock and severe ischemic change in organs 2.subacute DIC Rarely bleeding The evidence of DIC can be detected by laboratory examinations 3.chronic DIC,Stage of DIC,1.hypercoagulable stage 2.hypocoagulable stage 3.secondary fibrinolytic stage,第四節(jié) 臨床表現(xiàn),1.出血 凝血物質(zhì)消耗性 酶：破壞凝血因子 繼發(fā)性纖溶亢進 a FDP抗凝：競爭性抑制 a P聚 血管壁受損及溶栓,Consequences of DIC,1.disturbance of coagulation-bleeding (1)the consumption of clotting factors and platelets (2)the activation of fibrinolytic system (3)the production of fibrin degradation products (FDPs),2.休克 出血 回心血量 微血栓阻斷通路 CO 心泵功能 :心肌DIC BP 右心后負(fù)荷 : 肺DIC 外周阻力: 四個酶系統(tǒng)激活 A、B肽 擴血管物質(zhì) FDP (通透性) 激肽 C3a、C5a,2.disturbance of circulation-shock Microthromobus in capillariesand venules Blood returning decrease Cardiac muscle damage Cardiac output and blood volume reduce Effective circulating blood volume decrease Hypotension,3.栓塞 微血栓 器官 功能 BP 供血 障礙 4.溶血：微血管病性溶血性貧血,3.ischemic tissue damage-dysfunction of multiple organs Renal insufficiency Acute adrenal failure Pituitary necrosis Adult or acute respiratory distress syndrome (ARDS) Convulsion and coma,4.microangiopathic hemolytic anemia (MHA) characteristic morphologic abnormality of the red blood cells Twisted cells, crenated cells, triangular cells, helmet-shaped cells, and microspherocytes are seen on the blood smear.,Pathophysiological basis of laboratory diagnosis,1.detection of platelet count and its function 2.determination of clotting factors 3.determination of activity of fibrinolysis (1)thrombin time test (TT) (2)pl