Osteoporosis in Hemophilia

Abstract

Aim: The aim of this paper is to review the pathophysiology, risk factors, prevention and treatment of osteoporosis in persons with hemophilia (PWH). Study Design and Methodology: In a search of PubMed up to September 24, 2013 using as keywords "osteoporosis" and "hemophilia" the author found 61 references, of which only the 22 focused on the aim of this study were revised. Results: Prevention of osteoporosis in PWH is crucial. Risk factors are lack of hematological prophylaxis and development of an inhibitor (antibody) against the deficient coagulation factor, lack of exercise due to chronic pain and loss of joint function (hemophilic arthropathy), low body mass index and abnormal liver function because of viral infection (HIV, HCV). It has not been demonstrated that hemophilia has any effect on osteoclast development and/or osteoblast loss. Hemophilia does not impair mineral Ca/P/K mobilizations and metabolisms. Hemophilia does not have any general effects on the endocrine system. Substitution treatment with clotting factors does not interfere with the treatment of osteoporosis. Conclusion: Continuous primary hematological prophylaxis and rehabilitation and exercise are paramount in PWH. PWH over the age of 50 should have routine screening for detection of osteoporosis. In PWH prevention should encourage good habits (such as a diet adequate in calcium and vitamin D) and discourage harmful habits (such as tobacco, alcohol and immobilization). Osteoporosis is multi-facial. Osteoporosis ought to be promptly treated regardless of the underlying cause.