Abstract

Recurrent hemarthrosis in patients with hemophilia (PWH) results in chronic arthropathy requiring total joint replacement (TJR). This study aimed to compare the difference in TJR rate between patients with hemophilia A (HA) and hemophilia B (HB). A final total of 935 PWH (782 HA and 153 HB) without inhibitors were collected from the Taiwan’s National Health Insurance Research Database between 1997 and 2013. Demographics, clinical characteristics, and TJR rate were compared between the 2 groups. The annual use of clotting factor concentrate was not different between HA and HB groups (P = .116). The rate of comorbidities except for 29 PWH having HIV who were all in the HA group was also not different between the 2 groups. A total of 99 (10.6%) PWH had undergone 142 TJR procedures during the study period. All of them had received on-demand therapy. No difference was found in the cumulative incidence of TJR between HA and HB (P = .787). After adjusting for various confounders including age, pyogenic arthritis, and HIV infection, no increased risk of TJR was found in patients with HA versus Patients with HB (hazard ratio: 0.92, 95% confidence interval 0.54-1.58). This finding suggests that the rate of TJR between patients with HA and HB is not significantly different.

Highlights

  • Inherited hemophilia A (HA) and hemophilia B (HB) are X-linked bleeding disorders characterized by a deficiency or absence of clotting factor VIII (HA) or IX (HB)

  • Joint bleeding is the hallmark of severe hemophilia,[5] and recurrent episodes result in hemophilic arthropathy characterized by synovial hypertrophy and cartilage damage, leading to progressive joint damage and irreversible deformities.[6,7]

  • We identified that 99 (10.6%) of 935 patients with hemophilia (PWH) required total joint replacement (TJR), and no statistical difference in rate of TJR found between patients with HA (10.6%) and patients with HB (10.5%) between 1997 and 2013 (P 1⁄4 .954)

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Summary

Introduction

Inherited hemophilia A (HA) and hemophilia B (HB) are X-linked bleeding disorders characterized by a deficiency or absence of clotting factor VIII (HA) or IX (HB). Residual level of clotting factors in plasma defines the degree of hemophilia:

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