Suzuki Nobuaki
Department of Transfusion Medicine, Nagoya University Hospital.
Rinsho Ketsueki. 2020;61(7):779-784. doi: 10.11406/rinketsu.61.779.
Acquired coagulation inhibitors have become a popular area of research because they cause severe bleeding tendency in many patients. The use of acquired coagulation inhibitors requires rapid and precise diagnosis. Some acquired coagulation inhibitors show prolongation in the activated partial thromboplastin time (APTT) and/or prothrombin time (PT). To diagnose these disorders, mixing test is very useful. However, lupus anticoagulant related disorders, such as lupus anticoagulant hypoprothrombinemia syndrome (LAHPS), are difficult to diagnose because they are sometimes associated with a reduction in factor VIII and are thus difficult to distinguish from acquired hemophilia. Acquired factor XIII deficiency and acquired von Willebrand syndrome (AvWS) are easily overlooked because they show normal value in several patients with APTT and PT. Here I describe the diagnostic method for these disorders. In particular, five acquired coagulation inhibitors that appear to be clinically significant are studied.
获得性凝血抑制剂已成为一个热门的研究领域，因为它们在许多患者中会导致严重的出血倾向。获得性凝血抑制剂的使用需要快速、准确的诊断。一些获得性凝血抑制剂会使活化部分凝血活酶时间（APTT）和/或凝血酶原时间（PT）延长。为诊断这些疾病，混合试验非常有用。然而，狼疮抗凝物相关疾病，如狼疮抗凝物低凝血酶原血症综合征（LAHPS），很难诊断，因为它们有时与因子VIII降低有关，因此难以与获得性血友病区分开来。获得性因子XIII缺乏症和获得性血管性血友病综合征（AvWS）很容易被忽视，因为它们在一些APTT和PT正常的患者中表现为正常数值。在此，我描述这些疾病的诊断方法。特别对五种似乎具有临床意义的获得性凝血抑制剂进行了研究。
