Decreased Bone Mineral Density in Adult Patients with Hemophilia: Clinical Significance, Prevalence and Risk Factors

Introduction

Osteoporosis is widely recognized as a major health issue, and tremendous resources are spent screening, diagnosing, and treating this disease in postmenopausal women. According to the National Institutes of Health (NIH) Consensus Development Panel on Osteoporosis, 10 million people in the USA have osteoporosis and almost twice as many have decreased bone mass (1). Osteoporosis is defined as a decline in bone density and bone quality resulting in an increased risk for fragility fractures 1. It can be separated into primary osteoporosis resulting from gradual bone loss with aging and secondary osteoporosis due to a variety of medical disorders including chronic corticosteroid use, hypogonadism, alcoholism, and hyperthyroidism. The final common pathway for both primary and secondary causes of osteoporosis is increased bone resorption in relation to new bone formation.

Abstract

Osteoporosis is widely recognized as a major health problem, and considerable resources are spent screening, diagnosing, and treating this disease in postmenopausal women. Adults with hemophilia have unique factors that place them at an increased risk for developing osteoporosis including periods of immobilization after bleeds, impaired joint mobility that may limit weight bearing physical activity, and infection with Hepatitis C virus (HCV) and/or human immunodeficiency virus (HIV). Several publications report the prevalence of osteoporosis in adults with hemophilia to be in the range of 25% and 50%. The severity of hemophilic joint arthropathy appears to be the strongest predictor of significant bone loss in patients with hemophilia. The relative contribution of other risk factors, including HIV and HCV infection are less well defined. The clinical significance of decreased bone mineral density as measured by dual energy X-ray absorptiometry (DXA) to fracture risk in men, and in particular younger men, is also unclear. We recommend screening adult patients with hemophilia with documented fragility fractures, in those with severe joint arthropathy, and in those patients >50 years of age with additional risk factors such as HIV/HCV infection or low BMI. Adequate vitamin D and calcium intake, weight bearing physical activity, and interventions to maintain joint mobility are recommended for all hemophilia patients. The role of pharmacologic agents, including bisphosphonates, is untested, and they should be reserved for those with documented osteoporosis and fragility fractures or additional risk factors that place them at high risk for developing fragility fractures.

Keywords

hemophilia, joint arthropathy, osteoporosis, osteopenia, bone mineral density, fragility fracture