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Management of COVID-19-associated coagulopathy in persons with haemophilia
Steven W Pipe1, Radoslaw Kaczmarek2,3, Alok Srivastava4
1Departments of Pediatrics and Pathology, University of Michigan, Ann Arbor, MI, USA.
Insights
Managing COVID-19 coagulopathy in haemophilia patients requires tailored strategies. Recommendations focus on adjusting haemostatic treatments and thromboprophylaxis based on individual risk for bleeding and thrombosis.
Area of Science:
- Hematology
- Infectious Diseases
- Critical Care Medicine
Background:
- COVID-19 is linked to coagulopathy, increasing thrombosis risk, particularly in the pulmonary microvasculature.
- Managing COVID-19 coagulopathy in haemophilia patients presents unique challenges due to potential co-existing bleeding and thrombotic risks.
- These patients may require simultaneous antithrombotic and haemostatic therapies.
Purpose of the Study:
- To provide practical guidance for diagnosing and managing COVID-19 coagulopathy in individuals with haemophilia.
- To address the complexities of managing patients with inherited bleeding disorders during the COVID-19 pandemic.
Main Methods:
- Review of current literature on COVID-19's impact on haemostasis.
- Analysis of thromboprophylaxis guidelines for non-haemophilic patients.
- Extrapolation of management strategies for haemophilia patients based on clinical circumstances.
Main Results:
- Ambulatory patients should continue regular haemostatic treatment.
- Hospitalized patients on replacement therapy may require intensified factor concentrate prophylaxis alongside low molecular weight heparin (LMWH).
- Patients on emicizumab should continue treatment, potentially with added factor VIII and LMWH, based on bleeding and thrombosis risk.
Conclusions:
- Recommendations are derived from existing literature and aim to guide management in diverse clinical scenarios.
- Individual patient clinical status is crucial for tailoring treatment plans.
- Further clinical studies are needed to validate these management strategies.
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.
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