Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany.
Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Haemophilia. 2021 Feb;27 Suppl 3:5-13. doi: 10.1111/hae.14033. Epub 2020 Jun 1.
Acquired bleeding disorders can accompany hematological, neoplastic, autoimmune, cardiovascular or liver diseases, but can sometimes also arise spontaneously. They can manifest as single factor deficiencies or as complex hemostatic abnormalities. This review addresses (a) acquired hemophilia A, an autoimmune disorder characterized by inhibitory autoantibodies against coagulation factor VIII; (b) acquired von Willebrand syndrome in patients with cardiovascular disorders, where shear stress abnormalities result in destruction of von Willebrand factor; and (c) liver function disorders that comprise complex changes in pro- and anti-hemostatic factors, whose clinical implications are often difficult to predict. The article provides an overview on the pathophysiology, diagnostic tests and state-of-the-art treatment strategies.
获得性出血性疾病可伴随血液系统、肿瘤、自身免疫、心血管或肝脏疾病，但有时也可自发出现。它们可以表现为单一因子缺乏，也可以表现为复杂的止血异常。这篇综述讨论了以下内容：(a)获得性血友病 A，这是一种自身免疫性疾病，其特征是针对凝血因子 VIII 的抑制性自身抗体；(b)心血管疾病患者中的获得性血管性血友病，其中剪切应力异常导致血管性血友病因子的破坏；以及(c)肝脏功能障碍，包括促凝和抗凝因子的复杂变化，其临床意义通常难以预测。本文概述了其病理生理学、诊断测试和最新的治疗策略。
