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- Title
Clinical outcome of moderate haemophilia compared with severe and mild haemophilia.
- Authors
DEN UIJL, I. E. M.; FISCHER, K.; VAN DER BOM, J. G.; GROBBEE, D. E.; ROSENDAAL, F. R.; PLUG, I.
- Abstract
Information on outcome and treatment of patients with moderate haemophilia is scarce. In this study, we compared self-reported burden of disease in moderate haemophilia to severe and mild haemophilia. A nationwide questionnaire on bleeding pattern, treatment, impairment and quality of life was sent to 1567 Dutch patients with haemophilia. Out of 1066 respondents (response rate: 68%), 16% had moderate, 44% severe and 39% mild haemophilia. Median age was 36 years. Although overall outcome in moderate haemophilia was in between severe and mild haemophilia, moderate haemophilia patients did report a substantial burden of disease. The majority of patients with moderate haemophilia (73%) reported bleeds in the previous year; and a considerable proportion of moderate patients reported joint impairment (43%), chronic pain (15%), needed orthopaedic aids (24%) or were unemployed because of disability (27%). Within the group of moderate haemophilia patients, a large variation in bleeding pattern and outcome was observed. A quarter of patients with moderate haemophilia reported a more severe phenotype and intermittent use of prophylaxis. These patients reported frequent bleeding, with a median of eight bleeds per year, including two joint bleeds, and 68% reported joint impairment. In conclusion: Although outcome in moderate haemophilia is generally in between severe and mild haemophilia, moderate haemophilia patients reported a substantial burden of disease, and for more than 25% of patients with moderate haemophilia long term prophylaxis was implemented because of frequent bleeds.
- Subjects
HEMOPHILIA; HEMORRHAGE; CHRONIC diseases; CHRONIC pain; BLOOD diseases; HEMOPHILIACS
- Publication
Haemophilia, 2009, Vol 15, Issue 1, p83
- ISSN
1351-8216
- Publication type
Article
- DOI
10.1111/j.1365-2516.2008.01837.x