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

Authors: Pipe, SW.; Kaczmarek, R; Srivastava, A; Pierce, GF.; Makris, M; Hermans, C;

Affiliations: Departments of Pediatrics and Pathology, University of Michigan, Ann Arbor, MI, USA2Herman B Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, IN, USA ; Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Wroclaw, Poland ; Department of Haematology, Christian Medical College, Vellore, India; World Federation of Hemophilia, Montreal, QC, Canada ; Department of Infection, Immunity and Cardiovascular disease, University of Sheffield, UK ; Sheffield Haemophilia and Thrombosis Centre, Royal Hallamshire Hospital, Sheffield, UK ; Hemostasis and Thrombosis Unit, Division of Hematology, Cliniques universitaires Saint-Luc, Université Catholique de Louvain (UCLouvain), Brussels, Belgium ; Coagulation Products Safety Supply and Access Committee of the World Federation of Hemophilia, Montreal, QC, Canada ;

Publication: Haemophilia; 2021; 27. 41–48; March 2021

Abstract: 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-tic 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 thrombo-prophylaxis 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.