血管性血友病的诊断与管理

Diagnosis and management of von Willebrand's syndrome.

作者信息

Rick M E

机构信息

Hematology Service, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD.

出版信息

Med Clin North Am. 1994 May;78(3):609-23. doi: 10.1016/s0025-7125(16)30149-3.

DOI:10.1016/s0025-7125(16)30149-3

PMID:8170261

Abstract

von Willebrand's disease is the most common of the inherited bleeding disorders. It is caused by quantitative and/or qualitative abnormalities of von Willebrand factor, and it usually presents with bleeding from mucosal surfaces. The diagnosis is confirmed by measuring von Willebrand factor activity and antigen levels, factor VIII activity, and performing a multimer analysis of von Willebrand factor. Treatment may require plasma-derived concentrates, but can often be accomplished with DDAVP, a vasopressin analogue that causes transient release of von Willebrand factor from body storage sites.

摘要

血管性血友病是最常见的遗传性出血性疾病。它由血管性血友病因子的数量和/或质量异常引起，通常表现为黏膜表面出血。通过测量血管性血友病因子活性和抗原水平、因子VIII活性以及对血管性血友病因子进行多聚体分析来确诊。治疗可能需要血浆源性浓缩物，但通常使用去氨加压素（DDAVP）即可，它是一种血管加压素类似物，可使血管性血友病因子从体内储存部位短暂释放。