Low bone mass and hypovitaminosis D in haemophilia: A single‐centre study in patients with severe and moderate haemophilia A and B

Abstract

Introduction: Haemophilia (H) is frequently associated with a multifactorial reduction in bone mineral density (BDM), but little is known about possible differences between HA and HB according to their severity. Aim: To evaluate the association between low bone mineral density (BMD), 25-hydroxyvitamin D [25(OH)D] concentrations and bone turnover markers in patients with HA and HB younger or older than 50 years. Methods: In 78 patients <50 years and 33 patients >50 years with severe (S) or moderate (M) HA and HB, BMD was measured by dual-energy X-ray absorptiometry at femoral neck (FN) and lumbar spine and then correlated to annual bleeding rate (ABR), World Federation of Haemophilia orthopaedic joint scale (WFH score), 25(OH)D concentrations, parathyroid hormone (PTH), amino-terminal telopeptide of type 1 collagen (NTx), urinary pyridinolines, osteocalcin and bone-specific alkaline phosphatase. Results: Overall, a high prevalence of hypovitaminosis D was diagnosed. In patients <50 years, low FN-BMD was significantly more frequent in HA than in HB, while PTH, pyridinolines, ABR and WFH score were associated with H type and severity. In patients >50 years, similarly low FN-BMD was observed in HA and HB, while ABR and WFH score were associated with H type and severity, being milder in HB. Conclusions: Low bone mass is a frequent comorbidity in haemophilic patients of all ages, apart from those with MHB. Clinical and laboratory assessments confirm a higher bone impairment and faster bone resorption in HA compared with HB.

The Profile of Markers of Bone Turnover, Inflammation and Extracellular Neutrophil Traps on Bone Mass in Haemophilia and the Development of Haemophilic Arthropathy

“…Analysing the available medical documentation of patients, the authors also noted the prevalence of vitamin D deficiency in patients with haemophilia A and B—sufficient vitamin D levels were noted in 1/5 of patients, which is in accordance with the results of other studies [ 15 , 19 , 31 ]. According to researchers, vitamin D deficiency may be dictated by geographical location and not only by disease entity—most studies report that vitamin D deficiency is common in the European population [ 48 , 49 ].…”

“…Due to reports in the literature on the frequent occurrence of low bone mass in both adults and children with congenital haemophilia [ 15 , 19 , 31 , 32 , 33 ], the authors analysed the concentrations of selected markers of bone turnover in the venous blood of adult patients with haemophilia A and B. They demonstrated a statistically significant difference in osteocalcin values compared to a healthy control group.…”

“…However, the decrease in bone density depends on many factors and so far, no mechanism has been found for the development of osteoporosis and osteopenia in haemophilia. The literature has repeatedly attempted to find a relationship between haemophilia and bone loss [ 15 , 16 , 17 , 18 , 19 ], which would make it possible to reduce the incidence of osteoporosis by eliminating risk factors, and thus improve the quality of life of patients with haemophilia. It is well known that bone mass depends, inter alia, on physical activity, so it should correlate with the efficiency of the joints, but also with the possible fear of trauma and bleeding, which may occur in patients with congenital bleeding diathesis [ 20 ].…”

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

“…A specific effect of FVIII on BMD has been suggested by in vitro studies and the differences between HA and HB observed in Italian PWHs. 16,37 In our population, the comparison of HA and HB patients did not show clear differences, which is in line with an animal study 12 and a study on 14 HB patients in Iran, 38 which showed that HB also may lead to low BMD, not different from HA.…”

Low Bone Mineral Density in Hemophiliacs

“…Additionally, they delineated significantly reduced BMD of the femoral neck and an insignificant reduction in total hip BMD (25). Several more recent case-control studies also reported reduced BMD at these two skeletal sites, but did not find any significant differences in the lumbar spine (23,(26)(27)(28)(29)(30)(31).…”