Tiede Andreas, Susen Sophie, Lisman Ton
Department of Haematology, Hemostasis, Oncology, and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany.
Hemostasis and Transfusion Department, University of Lille, Lille University Hospital, Lille, France.
Haemophilia. 2024 Apr;30 Suppl 3:29-38. doi: 10.1111/hae.14995. Epub 2024 Apr 2.
Acquired bleeding disorders can develop in previously healthy people irrespective of age or gender but are particularly common in patients with certain underlying conditions. Here, we review recent advances in the management of acquired haemophilia A (AHA), acquired von Willebrand syndrome (AVWS), and patients with hemostatic abnormalities due to chronic liver disease (CLD). Patients with AHA can now benefit from prophylaxis with emicizumab, a therapeutic antibody that mimics the function of activated coagulation factor VIII. The treatment of AVWS remains challenging in many situations and requires careful consideration of the underlying condition. Haemostatic abnormalities in CLD are often compensated by proportional reduction in pro and anti-haemostatic factors resulting in sustained or even increased thrombin generation. Consequently, bleeding in CLD is rarely caused by haemostatic failure and infusion of plasma or coagulation factor concentrates may not be effective.
获得性出血性疾病可在既往健康的人群中发生，与年龄或性别无关，但在患有某些基础疾病的患者中尤为常见。在此，我们综述了获得性血友病A（AHA）、获得性血管性血友病综合征（AVWS）以及慢性肝病（CLD）所致止血异常患者管理方面的最新进展。AHA患者现在可受益于emicizumab预防治疗，emicizumab是一种模拟活化凝血因子VIII功能的治疗性抗体。在许多情况下，AVWS的治疗仍然具有挑战性，需要仔细考虑基础疾病。CLD中的止血异常通常通过促凝血和抗凝血因子成比例减少得到代偿，导致凝血酶生成持续甚至增加。因此，CLD出血很少由止血功能衰竭引起，输注血浆或凝血因子浓缩物可能无效。
