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1、出血性疾病总论Overview of Bleeding DiseaseDefinition Bleeding disorder refers to a group of diseases, that normal hemostatic mechanism disorder caused spontaneousbleeding, or excessive bleeding after minorinjury systems involved in hemostasisnVascular Spasm : the smooth muscle in blood vessel walls contrac
2、ts immediately, while the other hemostatic mechanisms become active.nPlatelet Plug Formation: the key stages of this process are: platelet adhesion, platelet release reaction, and platelet aggregationnBlood Coagulation- coagulation system, anticoagulation systemnfibrinolytic systemthe role of vascul
3、ar system in hemostasisnmechanism of vasoconstrictionnthe endothelium cells nevents following vascular injury Stimulate platelet adhesion - White thrombus formation Activation of coagulation system - solid red thrombus formation 血管在止血中的作用血管损伤血管收缩儿茶酚胺/5-HT/血管紧张素/加压素/内皮素/TXA2血流减弱血管内皮表达粘附分子配体内皮下成分暴露血小板
4、粘附、聚集、释放GPIb/vWFGPIIGPIIb b-III-IIIa a胶原/纤原ADP/TXA2/-TGPAF/Fg/vWF/Ca+合成、表达TFThrombin形成Fibrin形成血栓形成激活外源凝血系统Vessel wall abnormalities (vascular purpura)Congenital vascular wall abnormalities Hereditary telangiectasiaAcquired the vessel wall abnormalities immunityallergic purpura non-immune Infection:
5、bacteria, viruses, fungi, etc. Chemical : drugs, chemical toxins, etc. Metabolic: vitamin C deficiency, long-term use hormone Mechanical: trauma, pressure, etcSchematic diagram of platelet morphologythe role of platelets in hemostasisnplatelet adherence to collagen: GpIbnPlatelet aggregation：GpIIb/I
6、IIa complexnplatelet release reaction: ADP, PAF, TXA2nformation of platelet thrombosis and stabilization of the clot血小板在血小板在 止血中的作用止血中的作用血管损伤血管损伤胶原暴露胶原暴露血小板粘附血小板粘附血小板聚集血小板聚集胶原胶原VWFVWF血小板血小板GPIGPIb b-IX-IX纤维蛋白原纤维蛋白原GPIIGPIIb b-III-IIIa a血小板异常引起的出血举例血小板异常引起的出血举例血小板减少血小板粘附异常GPIb缺乏：巨大血小板病vWF缺乏：血管性血友病血小板
7、聚集异常GPIIb-IIIa缺乏：血小板无力症纤维蛋白原缺乏：血小板聚集障碍VWFGPIb-IX纤维蛋白原GPIIb-IIIaQuantitative and QualitativePlatelet DisordersQuantitative Disorders of PlateletsnThrombocytopenia Related to Decreased Production, Altered Distribution, Immune Effect, Consumption.nThrombocytosisInherited Qualitative Disorders of Platel
8、etsnDisorders of AdhesionnPlatelet Release DefectsAcquired Defects of Platelet FunctionVascular Disorders Leading to Platelet DysfunctionIn vivo coagulation cascadeIn vitro coagulation cascadeCoagulation factor disordersnHereditary Coagulation Disorders Hemophilia A (VIII factor deficiency) Hemophil
9、ia B (IX factor deficiency)nAcquired coagulation factor abnormalitiesCoagulation factor disorders AcquiredDecreased synthesisnSevere liver disease: factor VIII !nVitamin K antagonists: II, VII, IX, XIncreased consumption and destructionnFibrinolytic systemBleeding disorders clinical manifestationsAg
10、e GenderBleeding incentives Drugs, Surgery, trauma, Spontaneous bleeding, Infections etc.Bleeding frequency transient, recurrent or persistentFamily History CHARACTERISTICPATTERNSOFBLEEDINGINSYSTEMICDISORDERSOFHEMOSTASIS皮肤出血点（皮肤出血点（2mm2mm以内）以内）皮肤紫癜皮肤紫癜 (3(3 5mm)5mm) 口腔粘膜出血点口腔粘膜出血点皮肤大片淤斑皮肤大片淤斑关节血肿关节血
11、肿出血性疾病：实验室检查 反映血管壁功能的试验 常规试验 意义n毛细血管脆性试验 血管脆性、通透性n毛细血管镜 毛细血管襻形态、数量、结 构，血流状态 n病理活检 血管及周围组织的结构出血性疾病：实验室检查 反映血小板数量和功能的检查 常规试验 意义n血小板计数 血小板数量n血涂片 血小板形态n血小板聚集 聚集功能n血小板粘附 粘附功能n毛细血管脆性试验 血小板数量和功能，小血管结构功能，凝血n出血时间（出血时间测定器法） 同上出血性疾病：实验室检查 反映凝血因子含量和功能的试验 常规试验 意义n活化的部分凝血时间(APTT) 内源性凝血功能n凝血酶原时间(PT) 外源性凝血功能n凝血酶时间(
12、TT) 纤维蛋白原质或量、抗凝物质有无n纤维蛋白原nFDP,D-Dimer 纤维蛋白原、纤维蛋白原降解产物n n凝血因子抗原和活性 各凝血因子含量和功能Bleeding disorders: diagnostic procedures nBleeding disorder？nCause of disease ？ Vascular? platelet? Coagulation disorder? multiple factor?nLab tests to confirm the diagnosis 病例n男性，50岁,n临床表现：血尿、黑便、皮肤大片瘀斑数天，一月前类似发作史。自诉无诱因。n既往
13、无异常出血史，无类似家族史。无特殊药物服用史。n体格检查：轻度贫血貌，无慢性肝病面容，下肢大片瘀斑。 实验室检查会有哪些发现？n病例 实验室检查实验室检查nCBC：wbc11.37109/L,Hb77g/L,Bpc 287109/Ln肝肾功能：正常nPT、APTT 明显延长，TT正常，纤维蛋白原正常 ？n病例 维生素维生素K K依赖性凝血因子依赖性凝血因子缺乏缺乏n吸收不良综合征n阻塞性黄疸n长期服用肠道抗生素n服用华法林等双香豆素类抗凝药物n新型慢性抗凝血鼠药Bleeding Disorders: Treatment Principles nThe treatment for the causenSymptomatic treatment 1 hemostasis 2 replacement therapy 3 other treatments
