Department of Hematology, Graduate School of Medicine, Osaka Metropolitan University, Osaka.
Department of Laboratory Medicine, Tokyo Medical University, Tokyo, Japan.
Blood Coagul Fibrinolysis. 2023 Sep 1;34(6):419-422. doi: 10.1097/MBC.0000000000001243. Epub 2023 Jul 19.
Acquired hemophilia A (AHA) is a rare, life-threatening hemorrhagic disease caused by autoantibodies against factor VIII (FVIII), and bypassing agents (BPA) are used to control bleeding. However, some cases need a change of BPA or BPAs given sequentially or in combination for refractory bleeding. A 71-year-old man was admitted with subcutaneous hemorrhage. Laboratory investigations showed prolongation of activated partial thromboplastin time (APTT) and low-coagulation FVIII activity and FVIII inhibitor; we, therefore, diagnosed AHA. He was treated with recombinant factor VIIa (rFVIIa) BPA and prednisolone. However, his symptoms did not improve sufficiently, thus we switched BPA to activated prothrombin complex concentrate. Unfortunately, this was not effective and he suffered hemorrhagic shock. Therefore, we selected rFVIIa, with plasma-derived FVIIa and factor X (pd-FVIIa/FX) as combination therapy, and hemostasis was achieved without thrombosis. This case suggests that the combination of rFVIIa and pd-FVIIa/FX short-term can be well tolerated for refractory hemorrhage in AHA.
获得性血友病 A（AHA）是一种罕见的、危及生命的出血性疾病，由针对因子 VIII（FVIII）的自身抗体引起，旁路制剂（BPA）用于控制出血。然而，一些情况下需要更换 BPA 或顺序或联合使用 BPA 来治疗难治性出血。一名 71 岁男性因皮下出血入院。实验室检查显示活化部分凝血活酶时间（APTT）延长和低凝血因子 VIII 活性和因子 VIII 抑制剂；因此，我们诊断为 AHA。他接受了重组因子 VIIa（rFVIIa）BPA 和泼尼松龙治疗。然而，他的症状没有得到充分改善，因此我们将 BPA 更换为激活的凝血酶原复合物浓缩物。不幸的是，这没有效果，他出现了出血性休克。因此，我们选择了 rFVIIa，联合使用血浆源性因子 VIIa 和因子 X（pd-FVIIa/FX）作为联合治疗，达到了止血而没有血栓形成。这个病例表明，rFVIIa 和 pd-FVIIa/FX 的短期联合治疗可以很好地耐受 AHA 的难治性出血。
