Osteoporosis in hemophilia: what is its importance in clinical practice?

ABSTRACT

Introduction

The relationship between severe hemophilia and osteoporosis has been well established in the literature. However, although the importance of its prevention in order to reduce the risk of bone fractures has been reported, the importance of its treatment in clinical practice has not been well analyzed.

Areas covered

In this paper, a review of the available clinical and experimental information on osteoporosis in hemophilia has been performed, to better understand the relationship between hemophilia and osteoporosis. Prevention of osteoporosis in hemophilia should include primary hematological prophylaxis; a diet appropriate in calcium and vitamin D; a regular exercise program that includes aerobics, strength training and balance and flexibility activities; restriction of tobacco and alcohol use; and limitation of the duration of immobilization.

Expert opinion

Prevention of osteoporosis in hemophilic patients is paramount. However, it is noteworthy that there is only one publication on the treatment of osteoporosis in patients with hemophilia. Until further research is done on this topic, the existing recommendations for non-hemophilic patients should be followed. They include the use of antiresorptives (estrogens, selective estrogen receptor modulators, bisphosphonates, denosumab) and anabolic agents (teriparatide, abaloparatide, romosozumab). Further studies on the management of osteoporosis in patients with hemophilia are required.

KEYWORDS:

• Hemophilia
• osteoporosis
• experimental studies
• clinical studies
• prevention
• treatment

Article highlights

• It has been demonstrated that in hemophilia there is augmented osteoclastogenesis due to a deficiency of FVIII-VWF complex. This strengthens the resound for primary hematological prophylaxis in young patients with hemophilia beyond the preclusion of bleeding but also to promote healthy bones.

• It seems that vitamin D deficiency is an essential cause of reduced BMD in hemophilic children.

• Prevention of osteoporosis in hemophilia should include factor replacement therapy administered on a regular basis to preclude bleeding; a diet appropriate in calcium and vitamin D; a regular exercise program that included aerobics, strength training, and balance and flexibility activities; restriction of tobacco and alcohol use; and limitation of the duration of immobilization.

• It is noteworthy that there is only one publication on the treatment of osteoporosis in patients with hemophilia. Until more research on the topic is performed, the existing osteoporosis therapeutic agents for non-hemophilic patients should be used. They include antiresorptives (estrogens, selective estrogen receptor modulators, bisphosphonates, denosumab) and anabolic agents (teriparatide, abaloparatide, romosozumab).

• Taking into account that the bone phenotype develops already early in the life of PWH and most of the osteoporosis therapeutic agents should be precluded or not frequently utilized in children, due to their partly severe side-effects.

Declaration of interest

The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.