Abstract
Hemophilia is caused by a lack of antihemophilic factor(s), for example, factor VIII
(FVIII; hemophilia A) and factor IX (FIX; hemophilia B). Low bone mass is widely reported
in epidemiological studies of hemophilia, and patients with hemophilia are at an increased
risk of fracture. The detailed etiology of bone homeostasis imbalance in hemophilia
is unclear. Clinical and experimental studies show that FVIII and FIX are involved
in bone remodeling. However, it is likely that antihemophilic factors affect bone
biology through thrombin pathways rather than via their own intrinsic properties.
In addition, among patients with hemophilia, there are pathophysiological processes
in several systems that might contribute to bone loss. This review summarizes studies
on the association between hemophilia and bone remodeling, and might shed light on
the challenges facing the care and prevention of osteoporosis and fracture in patients
with hemophilia.
Keywords
hemophilia - factor VIII - factor IX - osteoporosis - osteogenesis - osteoclastogenesis
