DOI: 10.26650/jchild.2026.1997338 ISSN: 1308-8491

Arthropathy in Hemophilia Despite Prophylaxis: The Role of Cumulative Disease Burden Rather Than Bleeding Frequency

Halise Zeynep Genç, Başak Koç, Bülent Züfikar
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Introduction: Hemophilia A and B are inherited bleeding disorders characterized by recurrent bleeding episodes that can lead to progressive arthropathy, particularly in large joints. Although prophylactic factor replacement therapy reduces bleeding frequency, joint damage may continue to develop in some patients. The aim of this study was to evaluate the factors that determine the development of arthropathy in hemophilia patients receiving prophylaxis.Materials and Methods: This retrospective single-center study included 99 pediatric patients with hemophilia A or B who were followed between 2000 and 2018. Demographic characteristics, disease severity, duration of prophylaxis, follow-up duration, annual bleeding rate (ABR), annual joint bleeding rate (AJBR), and the presence of arthropathy were evaluated. Univariate logistic regression and correlation analyses were performed to identify the factors associated with arthropathy.Results: Of the 99 patients, 76.8% had hemophilia A and 23.2% had hemophilia B. Most patients with severe hemophilia received regular prophylactic treatment. Although baseline factor levels differed significantly according to disease severity, ABR and AJBR were comparable among patients receiving prophylaxis. Increasing age and longer follow-up duration were significantly associated with arthropathy, whereas ABR, AJBR, and factor level were not. Correlation analysis also demonstrated a significant positive association between follow-up duration and arthropathy, whereas no significant association was observed between bleeding parameters and joint damage.Conclusion: In patients with hemophilia receiving prophylaxis, the development of arthropathy appears to be associated with the cumulative burden of the disease and the duration of exposure rather than with the current bleeding frequency. These findings emphasize the importance of the early initiation and maintenance of prophylactic treatment in preventing long-term joint damage.

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