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Blood, 1 April 2002, Vol. 99, No. 7, pp. 2337-2341
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
The effects of postponing prophylactic treatment on long-term
outcome in patients with severe hemophilia
Kathelijn Fischer,
Johanna
G. van der Bom,
Eveline P. Mauser-Bunschoten,
Goris Roosendaal,
Robert Prejs,
Piet de Kleijn,
Diederick E. Grobbee, and
Marijke van den Berg
From the Van Creveldkliniek, Julius Center for General
Practice and Patient Oriented Research, Department of Radiology, and
the Department of Rehabilitation and Nutritional Sciences, University
Medical Center Utrecht, The Netherlands.
To prevent hemophilic arthropathy, prophylactic treatment of
children with severe hemophilia should be started before joint damage
has occurred. However, treatment is expensive, and the burden of
regular venipunctures in young children is high. With the aim of
providing information on starting prophylaxis on the basis of
individual patient characteristics, the effect of postponing prophylaxis on long-term arthropathy was studied in a cohort of 76 patients with severe hemophilia born between 1965 and 1985. The median
age at first joint bleed was 2.2 years (range, 0.2-5.8). Prophylaxis
was started at a median age of 6 years (interquartile range [IQR],
4-9), and the median annual clotting factor use on prophylaxis was 1750 IU/kg/y (31 IU/kg/wk). Hemophilic arthropathy was measured by the
Pettersson score (maximum, 78 points). At a median age of 19 years, the
median Pettersson score was 7 points (IQR, 0-17). After 2 decades of
follow-up, the Pettersson score was 8% higher (95% confidence
interval, 1%-16%) for every year prophylaxis was postponed after the
first joint bleed. This effect was independent of age at Pettersson
score, age at first joint bleed, and prophylactic dose used. In
conclusion, most patients have their first joint bleed after the age of
2 years. Patients who start prophylaxis soon after the first joint
bleed show little arthropathy in adulthood. The longer the start of
prophylaxis is postponed after the first joint bleed, the higher the
risk of developing arthropathy.
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