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Semin Thromb Hemost 2021; 47(01): 043-052
DOI: 10.1055/s-0040-1718890
Review Article

Mechanisms of Bone Remodeling Disorder in Hemophilia

Hanshi Wang
1   Department of Sports Medicine and Joint Surgery, The People's Hospital of China Medical University, Shenyang, People's Republic of China
,
Xizhuang Bai
1   Department of Sports Medicine and Joint Surgery, The People's Hospital of China Medical University, Shenyang, People's Republic of China
› Author Affiliations Funding This study was supported by the National Natural Science Foundation of China (grant No. 81671811). The funding source had no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; and in the decision to submit the article for publication.
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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


Publication History

Article published online:
23 December 2020

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