ArticleFrontiers in medicine2025
Impact of prophylaxis, inhibitors, and genetics on joint outcomes according to the IPSG-MRI score in hemophilia A, B and vWD type 3.
Article in Frontiers in medicine, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Introduction: Hemophilia A (HA) and hemophilia B (HB) are X-linked-bleeding disorders caused by deficiency of clotting factors VIII and IX, while von Willebrand disease (vWD) type 3 involves the lack of von Willebrand factor and FVIII. Chronic joint damage from recurrent bleeding is a serious complication. Aim: The aim was to investigate the association of prophylactical treatment, severity of the disease and joint outcome. Methods: In this retrospective, single-center study we evaluated joint health in 41 patients with HA, HB, and vWD type 3 who visited our outpatient clinic since 2000 using Magnetic resonance imaging (MRI) and applied the International Prophylaxis Study Group (IPSG) score. A total of 246 MRI images (knees, elbows, ankles) were analyzed in relation to disease severity, genetics, inhibitor-formation, and therapy. Results: Of 41 patients, 28 (68%) had severe HA or HB, 10 (24%) moderate, one (2%) mild, and two (5%) were vWD patients. 19 patients with severe HA/HB received primary prophylaxis. Inhibitors developed in 7 patients (17%), most of them had loss-of-function mutations. We observed hemophilic arthropathy in 7/39 (18%) hemophilia patients (all with severe HA/HB). Only one of the 19 patients receiving early prophylaxis developed arthropathy, in the context of inhibitor development. Minor changes (IPSG score 1-5) were observed in 20% of joints while 74% of joints showed no alterations (IPSG score 0). Only 6% of joints showed hemophilic arthropathy (IPSG score ≥ 8) with ankle joints most frequently affected (10%). Among vWD-patients, one exhibited minor changes; the other had no detectable joint damage despite vWF-inhibitor presence. Discussion: This study shows that the IPSG score is a suitable tool for assessing joint health in patients with hemophilia and vWD. Reduced joint damage was associated with early diagnosis, consistent prophylaxis, and therapy adherence.
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