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获得性血友病 A、同时发生的急性心肌梗死和紧急大手术:如何成功治疗使用 rpFVIII(Obizur®)的危急患者。

Acquired haemophilia A, concomitant acute myocardial infarction and urgent major surgery: How to successfully treat a critical patient with rpFVIII (Obizur®).

机构信息

Haemophilia Centre, Department of Medicine, University Hospital of Padua, Italy.

Haemophilia Centre, Department of Medicine, University Hospital of Padua, Italy.

出版信息

Thromb Res. 2020 Nov;195:125-127. doi: 10.1016/j.thromres.2020.07.012. Epub 2020 Jul 9.

Abstract

Association of acute myocardial infarction (AMI) and acquired haemophilia A (AHA) is extremely rare, and very difficult to treat, if the patient then presents several serious comorbidities and must undergo an urgent major surgery, the correct choice of haemostatic therapy is essential to avoid potentially life-threatening adverse events for the patient. The recombinant porcine FVIII (rpFVIII) is considered, together with the bypassing agents, as first-line therapy for the AHA, but compared to these it presents a reduced thromboembolic risk which makes it safer in patients who already present at the onset of haemorrhagic event an underlying cardiovascular disease. Haemorrhagic and thrombotic events are always to be taken into consideration in the case of surgery, but in the case of patients with concomitant acquired haemophilia the risk of having serious bleeding has further increase. Today there have been no reports of surgeries carried out under Obizur® coverage. We are reporting the successful case of an elderly critical patient with acquired haemophilia A and concomitant myocardial infarction, treated with rpFVIII and in which an urgent major surgery under coverage of this dug was performed for the first time.

摘要

急性心肌梗死(AMI)和获得性血友病 A(AHA)的关联极为罕见,且如果患者同时存在多种严重合并症并必须紧急进行大手术,则治疗非常困难,正确选择止血治疗方法对于避免危及生命的不良事件至关重要。重组猪 FVIII(rpFVIII)与旁路制剂一起被认为是 AHA 的一线治疗药物,但与这些药物相比,它具有降低的血栓栓塞风险,因此对于在出血事件发生时已经存在潜在心血管疾病的患者更安全。在手术情况下,始终需要考虑出血和血栓形成事件,但对于同时患有获得性血友病的患者,严重出血的风险进一步增加。目前尚无报道称在 Obizur®覆盖下进行手术。我们报告了首例成功的老年重症 AHA 合并心肌梗死患者的病例,该患者接受了 rpFVIII 治疗,并在 Obizur®覆盖下紧急进行了大手术。

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