出血性疾病 教学课件.ppt

1. 是否属于出血性疾病范畴 由外伤、手术等机械因素所致出血和某些疾病(如溃疡病、肝硬化)所致出血一般不列为出血性疾病。 2. 哪一类出血性疾病？ 血管性？血小板性？凝血障碍？其它？ 3. 数量异常还是质量异常？ 4. 先天性还是获得性？ 5. 确定诊断 诊断步骤 青年女性，无明显诱因反复全身多处皮肤瘀点瘀斑，月经量增多7年。多次查PLT40x109/L。自身抗体检查阴性。骨髓穿刺涂片细胞学检查：全片见巨核细胞620个，产板巨占8%，产板量少，血小板偶见。6天前行剖宫产术，术后24小时出血949ml，术后血小板维持在11-25x109/L，1天前出现血尿转入血液科。 病例分析 此病人有什么特点？ 是否为出血性疾病？ 哪一类出血性疾病？ 还需要做哪些检查？ 可能的治疗？ 假如你是接诊医生... * * Hemorrhagic是两个希腊词语组合而得来，“hema” or “haema” = blood;?rhegnumai?= to break forth（喷发，迸发），所以呢，很形象地解释了出血，即血液从血管中大量涌出。 * “hemostasis”comes from the?Ancient Greek?roots heme meaning blood, and stasis meaning halting; Put together means the halting of the blood. * * ?The physiologic haemostatic process is initiated by a disruption in the vascular wall and by the exposure of subendothelial components. Von Willebrand factor (VWF), binding to subendothelial collagen and to GPIb receptors on platelets, forms the first bridge between platelets and the vessel wall. The activation of platelets is induced initially by the interaction with collagen fibrils, present in the subendothelium, and with thrombin, formed locally owing to the exposure of tissue factor (TF), a strong direct initiator of the blood-clotting cascade present in extravascular tissue. Collagen and thrombin act on specific receptors on the platelet membrane: GPIa/IIa (or α2β1) and glycoprotein VI (GPVI) for collagen, and protease associate receptors (PARs) for thrombin. TxA2 is synthesized by activated platelets, from the arachidonic acid (AA) liberated from membrane phospholipids, through the cyclooxygenase (COX) pathway. Once formed, TxA2 diffuses across the membrane and activates other platelets by binding to its receptors (TPs) present on their surface. ADP is released from platelet granules and acts on two ADP receptors (P2Y1 and P2Y12), fostering the activation response and recruiting other platelets to the forming platelet plug. Platelet signalling events triggered by agonists mediate an increase in intraplatelet Ca2+ and a decrease in cAMP, leading to t