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Thank you for attending THSNA 2026. The virtual meeting is now closed.
Thank you for attending THSNA 2026. The virtual meeting is now closed.
Presentation Details
| Low-Dose Plasma-Derived von Willebrand Factor Concentrate for Surgical Prophylaxis in Ehlers-Danlos Syndrome with Normal VWF Profiles and Desmopressin Contraindications Joseph Stanco, Suchitra Acharya. Northwell Health, New Hyde Park, NY, and Cohen Children’s Medical Center, Queens, NY, USA |
Abstract
Background: Ehlers-Danlos Syndrome (EDS) is a connective tissue disorder characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. While hemostatic parameters, including von Willebrand factor (VWF) levels, are often normal in EDS patients, they frequently exhibit a disproportionate bleeding tendency due to vascular fragility and impaired platelet-collagen interaction. While desmopressin effectively elevates endogenous VWF levels, multiple contraindications limit its utility: age, medication interactions, tachyphylaxis, hyponatremia risk, cardiovascular disease, and non-responsiveness. The use of plasma-derived VWF (pdVWF) concentrate in EDS patients with documented normal VWF levels is not well-described, particularly regarding optimal dosing for surgical prophylaxis. Objectives: To evaluate the hemostatic efficacy and safety of low-dose (10–20 IU/kg) pdVWF concentrate for surgical prophylaxis in two patients with Hypermobile EDS (hEDS) with normal baseline VWF levels who were undergoing invasive procedures and had a contraindication to desmopressin. Methods: Retrospective chart review of two female patients with hEDS. Results: Patient 1: A 66-year-old female with hEDS diagnosed at age 16 following a ruptured ectopic pregnancy requiring multiple packed red blood cell transfusions. Her bleeding history included heavy menstrual bleeding and prolonged bleeding after dental extractions. While desmopressin provided adequate hemostatic coverage for parathyroidectomy in 2023, current contraindications included age, polypharmacy, and hyponatremia risk. The patient underwent cystoscopy with laser lithotripsy and ureteral stent exchange with prophylactic pdVWF at 20 IU/kg. Additional low-dose pdVWF was administered postoperatively for hemostatic support since antifibrinolytic therapy was contraindicated. Patient 2: 55-year-old female diagnosed with hEDS in 2024 presented with a lifelong history of hemorrhagic complications including recurrent epistaxis, iron-deficiency anemia secondary to heavy menstrual bleeding, and multiple episodes of post-procedural bleeding. Surgical history was notable for desmopressin failure: after a gastrectomy, she had significant bleeding that needed recombinant activated factor VII (rFVIIa) despite receiving both desmopressin and platelet transfusions. She also bled after a dilation and curettage (D&C), even though she received desmopressin before and after the procedure. Additional relevant history included platelet transfusion-associated urticaria and catheter-related deep vein thrombosis (DVT). Given documented desmopressin failure and prior thrombotic history, she received one dose of pdVWF at 10 units/kg prior to exploratory laparotomy and small bowel resection. Postoperative hemostasis was maintained with antifibrinolytic therapy alone for 7 days, without requirement for additional pdVWF administration. The procedure was completed without hemorrhagic complications, and no thrombotic events occurred during the postoperative period. Conclusion: Low-dose (10-20 units/kg) pdVWF concentrate effectively prevented peri - procedural bleeding in hEDS patients with normal VWF levels when desmopressin was contraindicated. This approach addresses the hemostatic challenges in EDS, potentially through VWF's role in platelet adhesion and connective tissue interaction. Further studies are warranted to establish optimal dosing protocols for this population.
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No part of this publication may be reproduced, distributed, or transmitted in any form or by any means, including photocopying, recording, or other electronic or mechanical methods, without the prior written permission of the author.