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凝血疾病 是指因凝血因子异常、血小板功能障碍或血管问题导致血液无法正常凝结或过度凝结的疾病,可分为 遗传性 和 获得性 两大类,具体类型多样且机制复杂。 凝血功能障碍是由凝血因子缺乏或功能异常引发的出血性疾病,分为遗传性与获得性两类。 遗传性多为单一凝血因子缺陷,如血友病;获得性常因维生素K缺乏、肝病或药物影响导致多因子缺乏。 这种疾病的患者在面部、嘴唇、口腔和鼻腔粘膜以及指尖和脚趾上有小的红到紫色的病变。 他们可能会经历鼻粘膜和胃肠道的反复出血,并且可能还会引起动静脉畸形的其他潜在严重后果。

常见的凝血系统疾病 血友病:主要由于缺乏凝血因子(A型缺乏因子VIII,B型缺乏因子IX)导致血液无法正常凝固,膝关节、肘关节和踝关节是最常见的出血部位。 凝血功能障碍的疾病包括遗传性凝血因子缺乏症,其症状有出血倾向、皮肤及黏膜瘀点、血尿、颅内出血和关节积血,建议患者及时就医。 缺乏维生素K:缺乏维生素K会导致凝血因子的合成减少,进而引起出血倾向,常见于营养不良、新生儿或某些消化道疾病患者。

凝血功能障碍性疾病主要包括血友病、假性血友病和血管内凝血等。 这些疾病通常会导致患者出现不同程度的出血问题。

HC-Ⅱ由肝脏合成,它与凝血酶以1:1 的 比例形成复合物、从而灭活凝血酶。 HC-Ⅱ的这一作用在肝素参与下可加快 1000倍。 它对因子Ⅹ的抑制作用缓慢, 且不被肝素所加快。 先天性HC-Ⅱ缺陷 少见,约占1%,获得性HC-Ⅱ缺陷见于 DIC和重型肝炎,且与AT-Ⅲ有平行关系。 当身体不能生成足够数量能帮助凝血、阻止出血的蛋白质时,身体就会出现凝血功能障碍性疾病。 这些蛋白质即所谓的凝血因子。 所有的凝血因子均在肝脏中生成。 肝脏需要维生素 K 来制造某些凝血因子。 有时,凝血异常会增加凝血风险(称为易栓症)。 凝血症是凝血功能障碍性疾病,由于多种原因导致凝血因子缺乏或凝血功能障碍,诱发出血不易止等表现,临床包括先天遗传性凝血功能障碍以及后天获得性凝血功能障碍,建议到血液内科就诊。

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