Abstract
ObjectiveTo review the current knowledge on bone health in patients with hemophilia A and the underlying pathogenetic mechanisms.Data SourcesOriginal research articles, meta-analyses, and scientific reviews.Data SynthesisAlready in childhood, patients with hemophilia A are prone to low bone mineral density, leading to osteopenia and/or osteoporosis. Initially associated with the life style of hemophilia, today we are faced with accumulating evidence that coagulation factor VIII is involved directly or indirectly in bone physiology.ConclusionUnderstanding the role of factor VIII and the mechanisms of decreased bone mineral density in hemophilia A is critically important, especially as non-factor replacement therapies are available, and treatment decisions potentially impact bone health.
Highlights
Prognosis and life expectancy for patients with hemophilia A have dramatically improved over the last decades
Similar results were obtained in 79 patients with severe hemophilia A, where significantly lower serum levels of tumor necrosis factor (TNF)-α, IL-10 and IL-12 were found in comparison to age-matched healthy controls [84], giving rise to the possibility that factor VIII (FVIII) deficiency leads to decreased bone-associated cytokine production
Patients with hemophilia are prone to develop low bone mineral density (BMD). Whether this bone phenotype is intrinsic to FVIII deficiency or secondary to it, is the subject of debate
Summary
To review the current knowledge on bone health in patients with hemophilia A and the underlying pathogenetic mechanisms. Data Sources: Original research articles, meta-analyses, and scientific reviews. Data Synthesis: Already in childhood, patients with hemophilia A are prone to low bone mineral density, leading to osteopenia and/or osteoporosis. Associated with the life style of hemophilia, today we are faced with accumulating evidence that coagulation factor VIII is involved directly or indirectly in bone physiology
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