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Anticoagulation strategies and recurrence of venous thromboembolic events in patients with sickle cell disease: a systematic review and meta-analysis

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Abstract

Sickle cell disease (SCD) results in many complications including an increased risk of developing venous thromboembolic events (VTEs) and an increased risk of mortality as a result. We conducted a systematic review using multiple databases to compare the efficacy of different anticoagulation in preventing recurrence, development of bleeding, progression of thrombus, and mortality in patients with SCD and a venous thrombotic event. Eight hundred seventy-one studies were screened and six studies were included. Among patients with SCD who experienced a VTE and were anticoagulated, the overall recurrence of VTE was 27.6% (95%CI 23.5–31.9). The overall progression to pulmonary embolism (PE) was 11.7% (95%CI 4.3–22.1). The overall bleeding rate was 14.1% (95%CI 7.8–21.9) and the overall mortality was 3.7% (95%CI 0.8–8.5). Based on observational studies, there did not appear to be differences between anticoagulant classes for the above adverse outcomes. Significant heterogeneity in the patient population and outcome measures limited the interpretation of the results. More studies, specifically randomized trials, are needed to help direct appropriate management of VTE’s in patients with sickle cell disease (PROSPERO ID: 236,208).

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Acknowledgements

We are grateful to the Children’s Hospital Foundation of Richmond that supported this study by providing access to Covidence and the funds to publish in an open access journal.

Funding

We are grateful to the Children’s Hospital Foundation of Richmond for the funds to publish in an open access journal.

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Authors and Affiliations

  1. Division of Pediatric Hematology and Oncology, Department of Pediatrics, Children’s Hospital of Richmond at Virginia Commonwealth University, Richmond, VA, USA

    Nadirah El-Amin & India Sisler

  2. Schoold of Medicine, Virginia Commonwealth University, Richmond, VA, USA

    Audra Iness

  3. Health Sciences Library, VCU Libraries, Virginia Commonwealth University, Richmond, VA, USA

    John W. Cyrus

  4. Division of Pediatric Critical Care Medicine, Department of Pediatrics, Children’s Hospital of Richmond at Virginia Commonwealth University, 1250 E Marshall St., Richmond, VA, 23298, USA

    Oliver Karam

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  1. Nadirah El-Amin
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Contributions

NE and OK conceptualized the research project, screened the articles, analyzed the data, and wrote the manuscript. IS and AI screened the articles for inclusion in the paper and edited the manuscript. JWC performed the initial search and edited the manuscript.

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Correspondence to Oliver Karam.

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El-Amin, N., Iness, A., Cyrus, J.W. et al. Anticoagulation strategies and recurrence of venous thromboembolic events in patients with sickle cell disease: a systematic review and meta-analysis. Ann Hematol 101, 1931–1940 (2022). https://doi.org/10.1007/s00277-022-04901-z

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  • DOI: https://doi.org/10.1007/s00277-022-04901-z

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