Abdulsalam Abbas Hashim, Ghiath Yusra, Alrahal Nidhal
Al-Mamoon University College, Baghdad, Iraq.
The National Center of Hematology, Baghdad, Iraq.
Hematol Oncol Stem Cell Ther. 2019 Dec;12(4):211-214. doi: 10.1016/j.hemonc.2018.05.006. Epub 2018 Jul 3.
Von Willebrand disease (VWD) is a bleeding disorder that results from decreased von Willebrand factor (VWF) activity <0.30 iu/mL. Therefore, the diagnosis of type 3 VWD in patients with bleeding requires finding a VWF:Ag and/or VWF:platelet ristocetin cofactor (RiCof) <0.03 iu/mL, no further testing is usually necessary. This is a cohort study that included 64 patients with type 3 VWD who were presented and diagnosed at the National Center of Hematology (NCH) from October 2014 to October 2016. In this study the sensitivity of VWF:Ag is only 78%, the sensitivity of VWF:RiCof is 92% of diagnosed cases. From our results it can be concluded that patients with type 3 VWD are usually presented with moderate/severe mucocutaneous bleeding that is associated with prolonged bleeding time test of >10 min and a family history of similar type of bleeding. This fact was frequently utilized to provisionally diagnose several members of the same family, forming a cohort of patients that is larger than the number of objectively-diagnosed patients included in this study, when they cannot afford to be all tested with VWF:Ag/VWF:RiCof.
血管性血友病（VWD）是一种出血性疾病，由血管性血友病因子（VWF）活性降低至<0.30 iu/mL所致。因此，对于有出血症状的患者，诊断3型VWD需要检测到血管性血友病因子抗原（VWF:Ag）和/或血管性血友病因子-血小板瑞斯托霉素辅因子（VWF:RiCof）<0.03 iu/mL，通常无需进一步检测。这是一项队列研究，纳入了2014年10月至2016年10月期间在国家血液学中心（NCH）就诊并确诊的64例3型VWD患者。在本研究中，VWF:Ag的敏感度仅为78%，VWF:RiCof的敏感度为已确诊病例的92%。从我们的结果可以得出结论，3型VWD患者通常表现为中度/重度黏膜皮肤出血，这与出血时间延长试验>10分钟以及类似出血类型的家族史有关。当同一家庭的几名成员负担不起全部进行VWF:Ag/VWF:RiCof检测时，这一事实常被用于对他们进行初步诊断，从而形成了一个比本研究中客观诊断的患者数量更大的患者队列。
