血友病 A 患者紧急出血的处理:关注 Emicizumab 的使用

V. Jiménez-Yuste, M. Álvarez‐Román, R. Berrueco, S. Bonanad, José M Calvo-Villas, Rebeca González-González, José R González Porras, Ramiro J Núñez-Vázquez, M. Rodríguez-López
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摘要

甲型血友病(HA)患者的治疗需要专业医护人员的知识和经验。然而,这些患者可能需要在转诊医院以外的紧急情况下接受治疗,而那里的医护人员并不了解血友病和/或新的创新治疗方法。本研究旨在开发一种简单实用的算法,供非专业治疗人员在紧急情况下使用,用于治疗接受或未接受埃米珠单抗预防性治疗的伴有或不伴有因子 VIII (FVIII) 抑制剂的血友病和出血患者。一组专家根据他们的临床经验商定了一个简单的算法,该算法操作简便,改编自以往的国际指南。建议的算法首先要识别患者、确诊 HA、使用埃米珠单抗预防和/或使用其他治疗方法。在稳定患者病情并对出血风险进行分层后,根据是否存在 FVIII 抑制剂对患者进行管理。没有 FVIII 抑制剂的患者应接受 FVIII 浓缩液治疗。剂量和随访取决于出血部位和严重程度。有 FVIII 抑制剂的患者最好接受重组活化因子 VII 作为旁路药物。在紧急情况下,有必要进行基本凝血检测、FVIII 评估和 FVIII 抑制剂检测。然而,由于埃米珠单抗可能会改变检测结果,因此在解释这些检测结果时应谨慎并适当选择。在紧急情况下,尤其是在使用新药治疗时,对 HA 患者的管理极具挑战性。非专业的凝血病医护人员对这种疾病的了解有限,因此需要一种算法来帮助他们做出明智的决定。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Management of Urgent Bleeding in Patients with Hemophilia A: Focus on the Use of Emicizumab
Management of patients with hemophilia A (HA) requires the knowledge and experience of specialized health care professionals. However, these patients may need to be attended in emergencies, outside the referral hospital, where health care professionals do not know about hemophilia and/or new innovative treatments. This study aimed to develop a simple and practical algorithm that could be used in emergency situations by nonspecialized treaters in HA and bleeding with or without factor VIII (FVIII) inhibitors under emicizumab prophylaxis. A group of experts agreed on a simple algorithm, easy to operate, adapted from previous international guidelines, and based on their clinical experience. The proposed algorithm starts with identifying the patient, confirming the diagnosis of HA, prophylaxis with emicizumab, and/or use of other treatments. After stabilizing the patient and stratifying the bleeding risk, the patient is managed according to the presence/absence of FVIII inhibitors. Patients without FVIII inhibitors should receive FVIII concentrate. Dose and follow-up depend on bleeding localization and severity. Patients with FVIII inhibitors should preferably receive recombinant activated factor VII as bypass agent. A basic coagulation assay, FVIII assessment, and FVIII inhibitors detection assays are necessary in an emergency. However, these tests should be interpreted with caution and appropriately chosen, as emicizumab may alter the results. The management of patients with HA is challenging in emergency situations, especially if they are treated with new agents. Nonspecialized in coagulopathies health care professionals have limited understanding of the disease, highlighting the need for an algorithm to assist them in making informed decisions.
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