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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 disease
2、 to all(%)Infection diseases 36.94Obstetric complications 24.81Malignancies 24.21Surgery and trauma 4.34Iatrogenic factor 1.45Other factors 8.25,DIC is characterized by the increasing loss of localization or compensated control in coagulation activation.,DIC pathogenesis is not just related to“coagu
3、lation 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：早期即出现肾、肺、大脑等器官功
4、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（1.0g/l=0；5：符合DIC；每日重复做检测如5：提
5、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 67PT 91 27 57APTT 91 42 57TT 83 60 70Fbg 22 100 65AT 91 40 70 FDP 100 67 87D-D 91 68 80破碎红细胞 23 73 512 联合试验（几个试验均为阳性）PT+APTT+TT 83 11 51 PT+APTT+Fbg 22 100 65PT+APTT+FDP 91 71 86FDP+D-D 91 94 95,D-二聚体在DIC患者明显增高,弥散性
6、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)
7、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
8、 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 where thrombosis predominates,heparin should be conside
9、red.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 rFVIIa.Heparin or LMWH is not suggested except for thrombo
10、-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 with chronic DIC or without active bleeding we do not suggest tra
11、nsfusion 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,SPI is recommended since it does not cause a worsening of bleeding.Bl
12、ood 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,UFH,and DS.,DIC and hyperfibrinolysis in acute promyelocytic leukemia,Zhaoyue
13、 WangJiangsu Institute of HematologyThe Affiliated Hospital of Soochow UniversityChina,Alterations of SFC,FDP and D-dimer in APL patients n SFC(mg/L)FDP(pg/L)D-Dimer(pg/L)Control 40 49.716.4 215.363.2 177.143.9DIC 15 958.6202.3*764.497.8*151662788*Non-DIC 35 316.9195.4*322.8175.2 23661135 DIC correc
14、ted 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 disease states，while ADAMTS13 deficiency could occur in sepsis-induced DIC.We report two patient
15、s 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.,女，43岁，因腹水住院检查。APTT 68.7s，PT 34.3s，TT 30.8s，Fg 1.2g/L，AT 41%，D二聚体7.1mg/L，血小
16、板54109/L。肝功能正常。B超发现胆管有一小包块。手术与病理检查证实为胆管癌广泛转移并发DIC。,男,64岁.皮肤瘀斑与血尿1月余,背部剧烈疼痛10天.有高血压史.全身皮肤粘膜瘀点瘀斑,背部大片瘀斑与皮下血肿.Hb 58 g/L,血小板72 109/L.APTT 46.2s,PT 23.6s,TT 40.7s,纤维蛋白原1.30g/L,D-二聚体 31.5 mg/L.CT示主动脉夹层瘤.行主动脉支架与颈部动脉置管,手术部位出血不止.,男，12岁，出水痘后10天全身大面积瘀斑，消化道呼吸道与泌尿道出血，反复颅内大出血，浓度昏迷。血小板24109/L，APTT、PT与TT明显延长，纤维蛋白原0
17、.21g/L，3P阳性，D-二聚体6.2mg/L。诊断DIC，大量输注血液、血浆与纤维后无效。后加用大剂量止血芳酸与抑肽酶后止血停止，很快苏醒。,Case 3 was a 13 year-old female.Since the age of 6 months,a hemihypertrophy on the right side of her body became gradually apparent.In the age of 10 years,she had a problem of hip dislocation,and then was effectively treated by
18、 open reductiona.In the age of 12 years,she suffered from a severe hematochezia.Digital subtraction angiography revealed abnormality of vascular structure in her ascending colon.However,abdominal operation did not find any Meckel diverticulum or vascular tumour in her small intestine.In the age of 1
19、3 years,she was admitted to our hospital because of continuing gum bleeding.,Alterations of DIC markers in two PS cases with giant hemangiomas Platelets APTT PT TT Fibrinogen AT D-dimer(109/L)(s)(s)(s)(g/L)(%)(g/L)Case 1Before splenectomy 71 61.7 20.1 22.8 0.6 50 19.1After splenectomy 110 37.2 14.4
20、18.2 2.91 83 3.22Case 2Before splenectomy 81 44.9 14.7 24.0 0.34 70.4 18.0 After splenectomy 138 40.1 14.5 18.2 3.06 98.6 1.95,A scoring system for diagnozing Proteus syndromeMacrodactyly and/or hemihypertrophy 5 pointsPlantar or palmar cerebriform hyperplasia 4 pointsLipomas/subcutaneous tumours 4
21、pointsEpidermal naevus 3 pointsMacrocephaly and/or skull exostosis 2.5 pointsmiscellaneous other minor abnormalities 1 pointA score of 13 or greater confirms its diagnosis.Our two patients scored 15.5 and 13 points,respectively,and met the criteria of Proteus syndrome diagnosis.,男，5岁，皮下大片瘀斑。WBC 8.71
22、09/L，RBC 3.671012/L，Hb 132 g/L，Plt 19109/L。初诊为ITP。,女，43岁，因卵巢囊肿住院手术。术后3天每天均有大血肿。APTT 86.7s，PT 120s，TT 15.8s，Fg 3.1g/L，血小板165109/L。拟诊为DIC。追问病史，因10年前瓣膜置换每日服用华法林（近半年为3mg/d），未作监测。,晚期肝硬化的凝血改变,男，52岁，晚期肝硬化肝功能衰竭，牙龈出血，皮肤少量瘀斑。APTT48.2s，PT31.4s，TT19.1s，纤维蛋白原0.87g/L，凝血酶原31%，因子42%，因子22.7%，因子104%，因子64%，因子37.8%，56.
23、6%。,VOD,女，22岁。ALL化疗后复发3次，接受来自母亲的半相合骨髓移植。术后第5天出现腹痛、腹胀、黄疸与肝肿大；体重每日增加10斤。全身皮肤粘膜广泛出血。T-BIL 84.5mol/L，ALT 1820 U/L，AST 2670U/L，LDH 2880 U/L。APTT 45.4s，PT 35.4s，TT 23.4s，Fg 1.03g/L，AT 38%，D二聚体3.18 mg/L，F:C 18%，F:C 4%，F:C 91%，F:C 23%，ADAMTS13 100%,Murine coagulation factor VIII is synthesized in endothelia
24、l cells,The primary cellular source of FVIII biosynthesis is controversial,with contradictory evidence supporting an endothelial or hepatocyte origin.Lman1 mutations result in combined deficiency of FV and FVIII,with levels of both factors reduced to 10%to 15%of normal in human patients.Endothelial
25、cells from multiple,but not all,tissues contribute to the plasma FVIII pool in the mouse.Blood.2014;123(24):3697-3705,女，2岁，外伤性肝破裂。入院后经大量输血、纤维蛋白原并给予重组活化因子与血凝酶等止血药物。经保守治疗后出血停止，恢复进食，一般情况良好，但凝血检查测不出，纤维蛋白原明显降低，抗体凝血酶水平与血小板计数正常。拟诊为DIC。,The diagnosis and treatment of DIC remain extremely controversial.Diagnosis and treatment DIC:Guidelines of the Italian Society for Haemostasis and Thrombosis,2012,由于医疗卫生的改善，近年来DIC的发病率明显下降,2004年为41.6/10万人，2010年已降至21.2/10万人，但死亡率没有显着的变化。Singh B.Chest.2013;143(5):1235,THANK YOU,
