Bone mineral density in haemophilia - a multicentre study evaluating the impact of different replacement regimens.

Abstract

The aim of this study was to investigate if prophylactic treatment in severe haemophilia impact on bone mineral densisty (BMD) in adults with haemophilia A/B. Subjects with haemophilia (n=120) underwent bone-density measurement and clinical data was collected. BMD in subjects with severe haemophilia on high-dose prophylaxis (n=41) was compared to BMD in subjects with mild haemophilia (n=33) and to severe haemophilia treated with intermediate-dose prophylaxis (n=32) or on-demand replacement therapy (n=14). Subjects with severe haemophilia on high-dose prophylaxis showed BMD at total hip comparable to subjects with mild haemophilia (median BMD 955.8 and 977.4mg/cm2 (P=.17), respectively). No difference in BMD was found related to type of prophylactic regimen (median BMD 955.8 and 942.4mg/cm2 , in high-dose and intermediate dose groups, respectively; P=.70). Subjects with severe disease treated on-demand had significantly lower BMD compared to subjects on a high-dose prophylactic regimen (median BMD 771.8 and 955.8mg/cm2 (P=.001), respectively). BMD decreased significantly with age, regardless of severity of haemophilia disease. In a multivariate analysis, adjusted for disease status and age, type of prophylactic regimen was not significantly associated with osteoporosis development. We show that BMD differs in persons with severe haemophilia on propylaxis as compared to those treated on-demand, but that type of prophylactic regimen does not reflect on BMD. The difference between treatment groups was mainly explained by an age difference between groups. However, patients on prophylaxis displayed a high degree of normal BMD not far from mild haemophilia at comparative age.