Management of COVID-19-associated coagulopathy in persons with haemophilia

Introduction 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. Objective We propose practical guidance for the diagnosis and management of COVID-19 coagulopathy in persons with haemophilia. Results 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. Conclusions 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.

Automated summary

COVID-19 associated coagulopathy in patients with hemophilia is complex, requiring a balance of antithrombotic and hemostatic treatments. Recommendations include continued hemostatic treatment, intensified prophylaxis with factor concentrates and LMWH based on bleeding risk, and possibly combining emicizumab with factor VIII and LMWH depending on individual patient needs. Dosage escalation of LMWH tailored to thrombosis risk may be considered, but lacks strong evidence support.