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Prevalence of pain in adult patients with moderate to severe haemophilia: a systematic review

  • Pia Ransmann EMAIL logo , Steffen Krüger , Thomas Hilberg , Thorsten Hagedorn and Nathalie Roussel



Patients with haemophilia (PwH) often suffer from joint pain due to repetitive haemarthroses and resulting arthropathy. Literature focuses so far on pain causes, diagnosis or treatment. A summary of prevalence rates, providing facts on the absolute occurrence of pain, is not sufficiently described so far. This review aimed to explore and systematically review different pain conditions, focussing on prevalence rates of pain in adult PwH.


A review of English articles using PubMed and Web of Science was conducted in February 2020. The search strategy included patients with haemophilia A or B suffering from pain. The articles were selected based on defined PICOS-selection criteria.


Out of 606 identified articles, 13 studies matched the given eligibility criteria and indicated pain prevalence rates. The weighted mean (WM) for the prevalence rate (varying timeframes) for chronic pain was 40% whereas for point prevalence the rate was WM=75%. Regarding pain intensity, findings of the EQ-5D-3L revealed moderate pain to be more present (61.0%) compared to extreme (11.6%). The main problem was the inconsistency of the definition of both acute and chronic pain as well as for prevalence types.


Pain is a major problem in patients with haemophilia. Pain therapy should be carried out taking into account the difference between bleeding-related or arthropathy-related causes of pain. In addition, the intensity and duration of pain should be recorded consistently to better monitor therapy and allow comparison with existing data.

Corresponding author: Pia Ransmann, Department of Sports Medicine, University of Wuppertal, Moritzstraße 14, 42117 Wuppertal, Germany, E-mail:
Thomas Hilberg and Nathalie Roussel contributed equally to this work.


The authors would like to thank Sietske De Keyser for her help in the literature search.

  1. Research funding: Authors state no funding involved.

  2. Author contributions: Pia Ransmann and Steffen Krüger and Nathalie Roussel performed the research and analysed the data. Pia Ransmann wrote the paper with support of Steffen Krüger, Thorsten Hagedorn, Thomas Hilberg and Nathalie Roussel.

  3. Competing interest: S. Krüger has received honoraria and travel support from Shire/Takeda and Swedish Orphan Biovitrum. S. Krüger was an employee at the Department of Sports Medicine during his work on the review and when the manuscript was written but is now employed by Takeda.

  4. Informed consent: This is not applicable as this article reviews prior literature.

  5. Ethical approval: This is not applicable as this article reviews prior literature.


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Received: 2021-09-06
Accepted: 2022-02-17
Published Online: 2022-03-16
Published in Print: 2022-07-26

© 2022 Walter de Gruyter GmbH, Berlin/Boston

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