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Thromb Haemost 2000; 84(06): 977-980
DOI: 10.1055/s-0037-1614159
Review Article
Schattauer GmbH

Endogenous Clotting Factor Activity and Long-term Outcome in Patients with Moderate Haemophilia

K. Fischer
1   From the Van Creveldkliniek, The Netherlands
2   Julius Center for General Practice and Patient Oriented Research, The Netherlands
,
J. G. van der Bom
1   From the Van Creveldkliniek, The Netherlands
2   Julius Center for General Practice and Patient Oriented Research, The Netherlands
,
E. P. Mauser-Bunschoten
1   From the Van Creveldkliniek, The Netherlands
,
G. Roosendaal
1   From the Van Creveldkliniek, The Netherlands
,
F. J. A. Beek
3   Department of Radiology, The Netherlands
,
P. de Kleijn
1   From the Van Creveldkliniek, The Netherlands
4   Department of Rehabilitation and Nutritional Sciences, University Medical Center Utrecht, The Netherlands
,
D. E. Grobbee
2   Julius Center for General Practice and Patient Oriented Research, The Netherlands
,
H. M. van den Berg
1   From the Van Creveldkliniek, The Netherlands
› Author AffiliationsThis study was partly supported by Zonne-projects of the WKZ, UMCU, Grant # 5.
Further Information

Publication History

Received 30 December 1999

Accepted after resubmission 10 July 2000

Publication Date:
13 December 2017 (online)

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Summary

In order to address the question of the optimum target level for prophylactic treatment of severe haemophilia patients, the association between endogenous clotting factor activity and outcome was studied in a cohort of 46 patients with moderate haemophilia. Data on treatment and outcome were collected annually.

Median follow-up was 8.0 years (range 1-26). Median joint bleed frequency was 1 per year and median radiological score according to Pettersson was 1 point (max. 78) at the age of 25 years. One percent increase in clotting factor level was associated with a 4 months later onset of joint bleeds (95% Confidence Interval (CI): 2-6 months) and a 16% lower Pettersson score (95% CI: 3-27%). No statistically significant effect of clotting factor activity on joint bleed frequency could be demonstrated.

These findings confirm that patients with moderate haemophilia experience only mild arthropathy, and provide evidence for a protective effect of higher clotting factor levels on joint damage.

Keywords

Moderate haemophilia - Pettersson score - haemarthrosis - arthropathy - prophylactic treatment
 

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