Osteoporosis in young haemophiliacs from western India

Abstract

Arthropathies and joint deformities in patients with severe hemophilia result in prolonged immobilization, reduced physical activity, and predispose them for osteoporosis. This can lead to an increasing tendency of bone fragility and fractures in patients after trivial trauma. The aim of this study was to find out (i) the prevalence of osteoporosis in hemophilia patients and (ii) the association of osteoporosis with hemophilic arthropathy and related restricted physical activity. In this case-control study, 50 consecutive severe hemophilia patients aged between 20 and 50 years were evaluated for osteoporosis with measurement of bone mass density (BMD) by a DEXA scan and values were compared with that of 50 sex matched normal healthy controls. Major joints of the limbs were evaluated to determine the extent of joint damage and related disability. Forty-two patients had severe hemophilia A and 8 patients severe hemophilia B (efficient factor activity < 0.01 U/ml). BMD values (gms/ cm 2 ) of lumbar spine and left hip of the patients were significantly lower than that of controls (0.825 vs. 0.939; P < 0.0001 and 0.725 vs. 0.938; P < 0.0001, respectively). The incidence of osteoporosis (T score: 22.5 or more) was significantly higher in hemophiliacs. Incidence of fractures in adult life was also significantly higher in hemophiliacs compared to controls (12% vs. 0%). There was statistically significant correlation between joint evaluation scores and BMD of hip, but not with that of the lumbar spine. There was no correlation between Hepatitis-C virus status and BMD of any site. This shows that development of osteoporosis is a significant problem in patients with severe hemophilia in this country. Hence appropriate preventive measures such as early treatment and adequate mobilization, exercises, encouragement to participate in sporting activities, early assessment of bone density, and administration of anti-osteoporotic therapy is recommended. Am. J. Hematol. 82:453-457, 2007. V V C 2007 Wiley-Liss, Inc.

Characteristics of Older Patient with Haemophilia

“…Our results, in a study where we evaluated 58 patients with haemophilia, showed reduced bone mass in 56% of patients 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.…”

“…Patients with haemophilia seem to achieve lower peak bone mineral mass when compared to healthy controls. In favour of this hypothesis, studies showed reduced bone mineral density among children with severe haemophilia (Barnes, 2004, Abdelrazik , 2007., Nair, 2007., Tlacuilo-Parra, 2008. The major impact on bone formation during childhood and adolescents has physical activity and, moreover, weight-bearing exercises.…”

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

“…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.…”

“…Only recently has it been demonstrated that hemophilia-induced osteoporosis could severely affect the morbidity of these patients, increasing the rate of fractures in adult life [10]. However, its pathogenetic mechanism has not been fully evaluated to date.…”

“…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.…”