Franchini Massimo, Targher Giovanni, Manzato Franco, Lippi Giuseppe
Servizio di Immunoematologia e Trasfusione-Centro Emofilia, Azienda Ospedaliera di Verona, Ospedale Policlinico, and Dipartimento di Scienze Biomediche e Chirurgiche, Università di Verona, Piazzale Ludovico Scuro, 37134 Verona, Italy.
Crit Rev Oncol Hematol. 2008 Jun;66(3):194-9. doi: 10.1016/j.critrevonc.2007.12.004. Epub 2008 Feb 19.
Acquired hemophilia A is an uncommon but potentially life-threatening hemorrhagic disorder caused by the onset of autoantibodies against coagulation factor VIII. Acquired hemophilia A is most frequently associated with autoimmune diseases, neoplasia, pregnancy and drug reactions but in approximately 50% of the cases no underlying disorder can be identified. A prompt diagnosis of this acquired bleeding disorder is essential for the appropriate management which is aimed to the control of hemorrhage and the suppression of inhibitor. Based on electronic and hand searches of the published literature, this systematic review examines the current knowledge on factor VIII autoantibodies associated with oncohematological disorders.
获得性血友病A是一种罕见但可能危及生命的出血性疾病，由针对凝血因子VIII的自身抗体的出现引起。获得性血友病A最常与自身免疫性疾病、肿瘤、妊娠和药物反应相关，但在大约50%的病例中，无法确定潜在疾病。对这种获得性出血性疾病进行及时诊断对于旨在控制出血和抑制抑制剂的适当管理至关重要。基于对已发表文献的电子和手工检索，本系统综述考察了与肿瘤血液学疾病相关的因子VIII自身抗体的现有知识。
