摘要
淀粉样变病可并发潜在的威胁生命的出血,其发病因素是异质性的和取决于淀粉样变的类型和器官累及的种类。轻链(AL)淀粉样变的患者获得性止血异常最重要的发病因素包括凝血因子的缺乏、过度纤溶和血小板功能异常。在其他类型的淀粉样变患者罕见获得性止血缺陷,已报道淀粉样沉积为异常出血表现的主要原因。淀粉样血管病血管脆性增加,损害血管收缩可促使出血。由淀粉样沉积引起实体器官破裂亦已有报道。由局部淀粉样沉积所致出血的治疗选择限于支持治疗,获得性止血缺陷可根据发病机制进行治疗。本文介绍淀粉样病患者出血的危险及有关的病理生理学、诊断和治疗的现代概念。
Amyloid diseases can be associated with potentially life -threatening hemorrhage. Pathogenetic factors contributing to the abnormal bleeding tendency in this setting are heterogeneous and depend on the type of amyloidosis and pattern of organ involvement. In patients with light -chain （AL） amyloidosis, acquired hemostatic abnormalities, including coagulation factor deficiencies, hyperfibrinolysis, and platelet dysfunction, can be regarded as the most important pathogenetic factors. In patients with other types of amyloidosis, acquired bemostatic defects are rare, and amyloid deposition has also been reported to be the main cause of abnormal bleeding manifestations. Amyloid angiopatby with increased fragility of blood vessels and impaired vasoconstriction may promote bleeding in this setting. Rupture of solid organs caused by amyloid deposition also was reported. Whereas therapeutic options in bleeding caused by local amyloid deposition are restricted to supportive measures and,in severe cases, surgery, acquired hemostatic defects may be treated according to the causative mechanism. In this review, we focus on bleeding risks in patients with amyloid diseases. Current concepts with regard to pathophysiology, diagnosis, and treatment are summarized and discussed.
出处
《现代肿瘤医学》
CAS
2009年第9期1810-1814,共5页
Journal of Modern Oncology
关键词
淀粉样变
出血
凝血因子缺乏
过度纤溶
血小板功能异常
淀粉样沉积
amyloidosis
bleeding
coagulation factor deficiencies
hyperfibrinolysis
platelet dysfunction
amyloid deposition
