The Bone Microarchitecture Deficit in Patients with Hemophilia Is Influenced by Arthropathy, Hepatitis C Infection, and Physical Activity

Abstract

Low bone mineral density (BMD) is common in patients with hemophilia (PWHs). The aim of the present study was to describe BMD and microarchitecture in PWHs in Northern Germany and to determine factors contributing to possible skeletal alterations. Demographic characteristics, BMD and microarchitecture, bone metabolism markers, and orthopaedic joint score (OJS) were assessed during routine check-ups. Areal BMD was assessed by dual-energy X-ray absorptiometry (DXA) at the hip and lumbar spine. Volumetric BMD and microarchitecture were quantified by high-resolution peripheral quantitative computed tomography at the distal radius and tibia. Eighty male PWHs (median age, 33 years; range, 18–77) were retrospectively analyzed, of whom 67 (84.0%) and 13 (16.0%) had hemophilia A and B, respectively. Fifty-four (68.0%), six (7.0%), and 20 (25.0%) patients had severe, moderate, or mild hemophilia, and 35 (44.0%) were hepatitis C virus (HCV) positive. DXA analysis revealed low BMD (Z-score ≤ − 2.0) in 27.5% of PWHs, and higher bone turnover values were associated with lower BMD. Bone microarchitecture was dominated by cortical deficits at the radius and trabecular deficits at the tibia. Cortical deficits at the radius were influenced by lower body mass index, low-grade inflammation, and treatment regimen (higher cortical thickness on primary prophylaxis). Trabecular alterations at the tibia were mainly associated with OJS and HCV status. A positive effect of self-reported sportive activity on BMD could be shown. In conclusion, our findings demonstrate that the site-specific microarchitectural deficit observed in PWHs is primarily negatively influenced by poor joint status, inflammation, HCV infection, and high bone turnover.

Diminished cortical bone density of long bones among children with haemophilic arthropathy

“…Trabecular alterations at the tibia were associated with orthopedic joint damage scores and hepatitis C virus status. 25 Taken together, these findings suggest that microarchitectural deficits start at an early age in haemophilia, and poor bone accrual and strength can be expected to worsen over time.…”

“…Cortical deficits at the radius were influenced by lower BMI, low‐grade inflammation, and treatment regimen: there was higher cortical thickness in patients on primary prophylaxis. Trabecular alterations at the tibia were associated with orthopedic joint damage scores and hepatitis C virus status 25 . Taken together, these findings suggest that microarchitectural deficits start at an early age in haemophilia, and poor bone accrual and strength can be expected to worsen over time.…”

Risk of low bone mineral density in patients with haemophilia: a systematic review and meta-analysis

“…Besides, a majority of patients in this Indian study exhibited chronic arthritis, which hampers regular exercise. Physical activity is known to promote BMD enhancement [ 37 ]. Calcium intake among Indians falls significantly below daily requisites due to dietary habits [ 38 ].…”

“…Lack of exercise affects bone mass and results in BMD not reaching its peak. Multiple studies [ 37 , 50 ] have suggested that sportive activity had a positive impact on BMD in PWH.…”