Desmopressin testing in von Willebrand disease: Lowering the burden

Autor: Jessica M. Heijdra, Ferdows Atiq, Wala Al Arashi, Quincy Kieboom, Esmee Wuijster, Karina Meijer, Marieke J.H.A. Kruip, Frank W.G. Leebeek, Marjon H. Cnossen, K. Fijnvandraat, R.A.A. Mathôt, S. Polinder, M. Coppens, R.Y.J. Tamminga, K. Meijer, B.A.P. Laros‐van Gorkom, P. Brons, S.E.M. Schols, F.J.M. van der Meer, H.C.J. Eikenboom, R.E.G. Schutgens, K. Fischer, F. Heubel‐Moenen, L. Nieuwenhuizen, P. Ypma, M.H.E. Driessens, C.M. Zwaan, I. van Vliet, P.W. Collins, R. Liesner, P. Chowdary, D. Keeling, J. Lock, H.C.A.M. Hazendonk, I. van Moort, T. Preijers, N.C.B. de Jager, M.C.H.J. Goedhart, L.H. Bukkems, M.E. Cloesmeijer, A. Janssen
Přispěvatelé: Paediatric Haematology, ARD - Amsterdam Reproduction and Development, Pharmacy, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, Vascular Medicine, ACS - Pulmonary hypertension & thrombosis, ACS - Amsterdam Cardiovascular Sciences, Graduate School, Other Research, Pediatrics, Hematology, Public Health, Erasmus MC other, Real World Studies in PharmacoEpidemiology, -Genetics, -Economics and -Therapy (PEGET)
Jazyk: angličtina
Rok vydání: 2022
Předmět:
Zdroj: Research and practice in thrombosis and haemostasis, 6(6):e12784. Wiley-Blackwell Publishing Ltd
Research and Practice in Thrombosis and Haemostasis, 6(6):e12784. Wiley-Blackwell Publishing Ltd
Research and practice in thrombosis and haemostasis, 6(6):e12784. Wiley
ISSN: 2475-0379
Popis: Background: Individuals with von Willebrand disease (VWD) require desmopressin testing because of interindividual response differences. However, testing is burdensome, while not all patients may need extensive testing.Objectives: To provide von Willebrand factor (VWF) cutoffs that predict desmopressin nonresponse and thereby identify individuals who do not need extensive testing in a retrospective cohort. We validated these cutoffs in a prospective cohort.Patients and Methods: We included 376 patients (Type 1 VWD with VWF activity [VWF:Act] Results: All individuals with Type 1 VWD and Type 2 VWD, respectively, with baseline VWF:Act 0.34 IU/ml or greater or 0.28 IU/ml or greater were responders. In individuals with T1 VWF:Act ≥0.89 IU/ml (Type 1 VWD) or T1 VWF:Act 1.10 IU/ml or greater (Type 2 VWD), response remained at T4.Conclusion: Desmopressin testing is not needed when lowest historical VWF:Act is 0.30 IU/ml or greater. In patients with Type 1 VWD who require testing, measurements after T1 are often not needed. In patients with Type 2 VWD who require testing, we advise performing T1 and T4 measurements.
Databáze: OpenAIRE
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