罕见于 3 型 VWD 患者;通常见于有大量血管性血友病因子 (VWF) 基因缺失的患者中。可能引起对外源性 VWF 的严重过敏反应。有报道显示,持续输注高纯度因子 VIII 或重组活化因子 VIII,可成功治愈此类患者的出血。[18]Laffan MA, Lester W, O'Donnell JS, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol. 2014 Nov;167(4):453-65.http://onlinelibrary.wiley.com/doi/10.1111/bjh.13064/fullhttp://www.ncbi.nlm.nih.gov/pubmed/25113304?tool=bestpractice.com[20]Rodeghiero F, Castaman G. Treatment of von Willebrand disease. Semin Hematol. 2005;42:29-35.http://www.ncbi.nlm.nih.gov/pubmed/15662613?tool=bestpractice.com
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