Reduced Bone Mineral Density in Hemophilia.

Rationale: Hemophilias are hereditary bleeding disorders usually presenting with muscloskeletal bleeding (for example, hemarthroses, synovitis and hematomas). Recently it has been suggested that patients with hemophilia are at increased risk of osteoporosis. The arthropathy associated with hemophilia, along with the treatments for human immune defeciency virus (HIV) and hepatitis C virus (HCV) may predispose affected individuals to osteoporosis and pathologic fractures.

Methods: This was a retrospective study that included unselected male patients aged 18 yrs old and over with severe hemophilia managed and followed in the Hamilton-Niagara Regional Hemophilia Program. All patients have been recommended to have bone mineral density studies (BMD). BMD was performed using dual x ray absorptiometry.

Results: There were 19 patients, mean age 29 years, range 18–43. Prophylactic treatment with recombinant factors was received by 12/19 patients (63%); HCV infection was present in 14/19 (73%) and HIV in 6/19 (31%) patients, the latter all receiving HAART treatment. A reduced BMD (osteopenia) as defined by the World Health Organization was detected in 42% of patients (5/13 or 38% of Hemophilia A and 3/6 or 50% of Hemophilia B participants). Among those who received prophylactic recombinant factors, 5/12 had reduced BMD. There was a trend towards decreased BMD in patients with HIV or HCV.

Conclusions: Individuals with severe hemophilia are at increased risk of having low bone mineral density. Those with coexistent HIV and HCV infections may be at higher risk. There was no clear correlation in this small series between BMD and age, and the use of prophylactic factor concentrate. Further study is required to obtain a more precise prevalence of disease, and clarification of the risk factors; however, a high prevalence of osteopenia in such a young cohort of individuals is concerning and estimation of BMD in all individuals with severe hemophilia is advised.