Osteoporosis in haemophilia – an underestimated comorbidity?

Abstract

A relationship between haemophilia and osteoporosis has been suggested, leading to the initiative for a larger study assessing this issue. Bone mineral density (BMD) was measured by osteodensitometry using dual energy X-ray absorptiometry (DEXA) in 62 male patients with severe haemophilia A; mean age 41 +/- 13.1 years, mean body mass index (BMI) 23.5 +/- 3.6 kg m(-2). Using the clinical score suggested by the World Federation of Hemophilia, all patients were assessed to determine the severity of their arthropathy. A reduced BMD defined as osteopenia and osteoporosis by World Health Organization criteria was detected in 27/62 (43.5%) and 16/62 (25.8%) patients, respectively. Fifty-five of sixty-two (88.7%) patients suffered from haemophilic arthropathy. An increased number of affected joints and/or an increased severity were associated with lower BMD in the neck of femur. Pronounced muscle atrophy and loss of joint movement were also associated with low BMD. Furthermore, hepatitis C, low BMI and age were found to be additional risk factors for reduced BMD in the haemophiliac. Our data shows that in haemophilic patients osteoporosis represents a frequent concomitant observation. The main cause for reduced bone mass in the haemophiliac is most probably the haemophilic arthropathy being typically associated with chronic pain and loss of joint function subsequently leading to inactivity. Further studies including control groups are necessary to elucidate the impact of comorbidities such as hepatitis C or HIV on the development of osteoporosis in the haemophiliac.

Increased bone resorption is implicated in the pathogenesis of bone loss in hemophiliacs: correlations with hemophilic arthropathy and HIV infection

“…Among them, more than a half (55.6%) had osteoporosis and the rest (30.5%) had osteopenia. Gallacher et al first reported a reduced BMD in 19 hemophilia patients [8], and recently, similar results had also been reported in a larger study with 62 hemophilia patients [9]. In that study, 70% of the patients had reduced BMD in the FN by DEXA evaluation.…”

“…The results from the studies that have investigated the impact of hepatitis C on osteoporosis of hemophilia patients are conflicting. In two studies [10,17], no difference in the severity of osteoporosis between HCV-positive and HCVnegative patients was observed, while in the study of Wallny et al [9], a significant difference in mean values of BMD was reported in the two groups. Almost all of the patients of our cohort were HCV-positive, and this could be considered as a factor implicated in the high incidence of osteoporosis observed in our study.…”

“…Prolonged immobilization and lack of weight-bearing exercise, as well as comorbid infections [hepatitis C virus (HCV) and human immunodeficiency virus (HIV)], are suggested as the main factors contributing to the pathogenesis of osteoporosis in hemophilia. Although there are a few studies where the prevalence of osteoporosis was investigated [9,10], the exact pathogenetic mechanism has not yet been fully clarified.…”

Characteristics of Older Patient with Haemophilia

“…Those results are comparable with findings of Nair, who described reduced bone mass in 50% of patients with severe haemophilia (Nair, 2007). On the other hand, some authors showed reduced bone mass in 86% patients with severe and moderate haemophilia (FVIII<3%) (Katsarou, 2009), and in 70% of patients with severe haemophilia (Gerstner, 2009, Wallny, 2007. The reason for difference between those results is uncertain.…”

“…They have to do functional training (hydrotherapy, walking climbing stairs, cycling, etc). Osteopenia and osteoporosis can cause increased number of serious injuries and fracture after falls (Wallny, 2007). Painful haemophilic arthropathy with reduced mobility and lack of activity may lead to a reduction of bone mass.…”