Departments of Pediatrics and Pathology, University of Michigan, Ann Arbor, MI, USA.
Herman B Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, IN, USA.
Haemophilia. 2021 Jan;27(1):41-48. doi: 10.1111/hae.14191. Epub 2020 Nov 20.
The SARS-CoV-2 coronavirus-induced infection (COVID-19) can be associated with a coagulopathy mainly responsible for pulmonary microvasculature thrombosis and systemic thromboembolic manifestations. The pathophysiology and management of the COVID-19 coagulopathy are likely more complex in patients with inherited bleeding diseases such as haemophilia. These individuals might indeed present with both bleeding and thrombotic complications and require simultaneous antithrombotic and haemostatic treatments.
We propose practical guidance for the diagnosis and management of COVID-19 coagulopathy in persons with haemophilia.
Continuation of regular haemostatic treatment is recommended for ambulatory patients. For patients requiring hospital admission and on replacement therapy with factors VIII or IX concentrates, prophylaxis with concentrates should be intensified according to the risk of bleeding complications and associated with prophylactic doses of LMWH. For patients on nonreplacement therapy, emicizumab should be continued and possibly combined with factor VIII and prophylactic doses of LMWH depending on the risk of bleeding and thrombosis. Dose escalation of LMWH tailored to the risk of thrombosis can be employed but not supported by evidence.
These practical recommendations are based on the current literature on COVID-19 with its impact on haemostasis, indications and modalities for thromboprophylaxis mainly in nonhaemophilic patients and how that is likely to affect persons with haemophilia in different circumstances. They will need to be tailored to each patient's clinical status and validated in future studies.
由 SARS-CoV-2 冠状病毒引起的感染（COVID-19）可引起凝血功能障碍，主要负责肺微血管血栓形成和全身血栓栓塞表现。COVID-19 凝血功能障碍的病理生理学和管理在遗传性出血性疾病（如血友病）患者中可能更为复杂。这些患者实际上可能同时出现出血和血栓并发症，需要同时进行抗血栓和止血治疗。
我们提出了血友病患者 COVID-19 凝血功能障碍的诊断和管理实用指南。
建议对门诊患者继续进行常规止血治疗。对于需要住院治疗且正在接受因子 VIII 或 IX 浓缩物替代治疗的患者，应根据出血并发症风险和相关 LMWH 预防性剂量来强化浓缩物预防。对于接受非替代治疗的患者，应继续使用emicizumab，并根据出血和血栓形成的风险，可能与因子 VIII 和预防性 LMWH 联合使用。可根据血栓形成风险调整 LMWH 的剂量，但目前尚无证据支持。
这些实用建议基于 COVID-19 对止血的影响的当前文献，以及主要在非血友病患者中进行血栓预防的指征和方式，以及这可能如何在不同情况下影响血友病患者。它们需要根据每个患者的临床状况进行调整，并在未来的研究中进行验证。
