BMD Reduced, but Stable Throughout Course of Hemophilia, Say Findings

Nearly half of patients with hemophilia have decreased bone mineral density (BMD), suggest study findings published in Hamostaseologie, which also showed that levels remain consistent throughout the course of the bleeding disorder. Prior to this study, explained the researchers, data on the long-term course of osteoporosis in these patients have been lacking.

Across the 33 adult patients in the retrospective study, 48.5% had osteopenia and 21.2% had osteoporosis at the last follow-up. In line with previous studies, the researchers observed lower vitamin D levels among their patients, suggesting that screening for the deficiency and supplementing with vitamin D may be warranted.

“In general, a vitamin D deficiency is a frequently mentioned influencing factor on BMD. With vitamin D being essential for the bone metabolism, a lack of it results in an increased risk for osteoporosis,” commented the researchers. “Within our cohort study, 78.8% of the patients suffered from a vitamin D deficiency (< 30 ng/mL). Kempton et al reported reduced vitamin D levels in one-third, up to one-half of the patients with hemophilia. By contrast, according to Rabenberg et al, only 38.4% adults in the standard German population (38.3% of all men) suffer from a vitamin D deficiency.”

Each patient received at least 2 bone density measurements with a minimum range of 10 years, with results not changing significantly from one point of time to the next. The researchers noted that because of the long observation period, not all dual-energy x-ray absorptiometry scans were performed with the same device.

Almost half of the patients received osteoporosis-based treatment, including vitamin D and calcium. However, receiving anti-osteoporotic treatment, whether basic or specific, was associated with a significant change in BMD.

Meanwhile, there was a significant positive correlation between body mass index and BMD and significant negative correlation between Gilbert score of joint health and t-score for bone density, the latter having been documented previously. Based on the finding, the researchers suggest practices consider using Gilbert score for pre-estimating the benefit of a more invasive osteoporosis diagnostic approach.

“Consumption of nicotine and alcohol, despite being well-known risk factors for osteoporosis, did not affect bone mass in a statistically significant way,” explained the researchers. “This is also true for our patients’ most frequent comorbidities such as hypertension, type 2 diabetes, dyslipidemia, and liver cirrhosis. Even repetitive local or systemic cortisone therapy did not affect patients’ BMD in the course of time.”

Morbidity rating also showed no effect on bone mass.

Based on their findings, the researchers flagged a need for further research to assess the reason for osteopenia in patients with hemophilia.

Reference

Strauss A, Muellejans P, Koob S, et al. Osteoporosis remains constant in patients with hemophilia—long-term course in consideration of comorbidities. Hamostaseologie. Published online March 2, 2023. doi:10.1055/a-1972-8983