Abstract Number: PB0642
Meeting: ISTH 2021 Congress
Background: Joint arthropathy can significantly impact the quality of life of patients with hemophilia, and often leads to the need for joint replacement. Early use of prophylactic factor replacement can reduce or prevent arthropathy; however, some joint bleeds may still occur. rVIII-SingleChain is a long-acting novel recombinant FVIII (rFVIII) with improved pharmacokinetic and pharmacodynamic properties compared with standard-acting rFVIII.
Aims: To describe the case of a 49-year-old male with severe hemophilia A, hemophilic arthropathy, and target joints who switched from prophylaxis with plasma-derived von Willebrand factor (VWF)/FVIII (pdVWF/FVIII) to rVIII-SingleChain.
Methods: A patient treated prophylactically with pdVWF/FVIII twice-weekly since March 2018 was referred for orthopedic consultation and assessed using HEAD-US. He presented with a high annualized bleeding rate (ABR) and annualized joint bleeding rate (AJBR) despite high treatment adherence. He was deemed a good candidate for switching to rVIII-SingleChain to reduce his bleeding frequency.
Results: In February 2018, assessment using HEAD-US showed damage to the articular surfaces in the cartilage in both ankles and both elbows and in the bone in both ankles and the left elbow; synovitis was observed in both ankles. In July 2019, the patient switched to rVIII-SingleChain prophylaxis every four days. Compared with baseline, after 1 year of treatment, adherence to the regimen was improved (92.9% vs. 100%) and both ABR and AJBR significantly improved (4.61 vs. 0 and 4.61 vs. 0, respectively). A further assessment using HEAD-US in November 2020 showed that synovitis was no longer present in the right ankle. All target joints were considered resolved following the switch. The patient is satisfied with his treatment and has not required any extra infusions despite an active lifestyle.
Conclusions: This case report demonstrates an improvement in joint health following the switch to rVIII-SingleChain in a patient with severe hemophilia A and significant joint damage.
To cite this abstract in AMA style:Benítez Hidalgo O, Martinez Garcia MF, Miranda Pinzon N, Ibañez Gomez E, Blazquez Gomez N, Suito Alcantara M. Joint Health Improvement Following a Switch to Prophylaxis with rVIII-SingleChain: A Patient Case [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/joint-health-improvement-following-a-switch-to-prophylaxis-with-rviii-singlechain-a-patient-case/. Accessed November 27, 2021.
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