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CC BY 4.0 · TH Open 2023; 07(01): e76-e81
DOI: 10.1055/a-2008-4367
Case Report

Treatment of Acquired von Willebrand Disease due to Extracorporeal Membrane Oxygenation in a Pediatric COVID-19 Patient with Vonicog Alfa: A Case Report and Literature Review

Lars Heubner 
1   Department of Anesthesiology and Intensive Care Medicine, University Hospital Carl Gustav Carus at the Technische Universität Dresden, Dresden, Germany
,
Karolin Trautmann-Grill
2   Department of Internal Medicine I, University Hospital Carl Gustav Carus at the Technische Universität Dresden, Dresden. Germany
,
Oliver Tiebel
3   Institute of Clinical Chemistry, University Hospital Carl Gustav Carus at the Technische Universität Dresden, Dresden. Germany
,
Martin Mirus
1   Department of Anesthesiology and Intensive Care Medicine, University Hospital Carl Gustav Carus at the Technische Universität Dresden, Dresden, Germany
,
Andreas Güldner
1   Department of Anesthesiology and Intensive Care Medicine, University Hospital Carl Gustav Carus at the Technische Universität Dresden, Dresden, Germany
,
Axel Rand
1   Department of Anesthesiology and Intensive Care Medicine, University Hospital Carl Gustav Carus at the Technische Universität Dresden, Dresden, Germany
,
Peter Markus Spieth
1   Department of Anesthesiology and Intensive Care Medicine, University Hospital Carl Gustav Carus at the Technische Universität Dresden, Dresden, Germany
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Abstract

Acquired von Willebrand disease (aVWD) is frequently observed in patients with the need for extracorporeal membrane oxygenation (ECMO). aVWD can be treated by plasma-derived concentrates containing factor VIII (FVIII) and/or von Willebrand factor (VWF) and recombinant VWF concentrate as well as adjuvant therapies such as tranexamic acid and desmopressin. However, all of these therapeutic options possibly cause thromboembolism. Therefore, the optimal treatment remains uncertain. This report presents a case of a 16-year-old patient suffering from severe acute respiratory distress syndrome due to coronavirus disease 2019 with the need of ECMO support. Our patient developed aVWD under ECMO therapy characterized by loss of high-molecular-weight multimers (HMWM) and severe bleeding symptoms following endoscopic papillotomy due to sclerosing cholangitis. At the same time standard laboratory parameters showed hypercoagulability with increased fibrinogen level and platelet count. The patient was successfully treated with recombinant VWF concentrate (rVWF; vonicog alfa; Veyvondi) combined with topic tranexamic acid application and cortisone therapy. rVWF concentrate vonicog alfa is characterized by ultra-large multimers and absence of FVIII. Patient could be successfully weaned from ECMO support after 72 days. Multimer analysis 1 week after ECMO decannulation showed an adequate reappearance of HMWM.

Keywords

acquired coagulation disorders - von Willebrand disease - extracorporal circulation

Authors' Contributions

L.H. – clinical management, conducting research, and drafting the paper including critical revisions. K.T.G. – clinical management and revising the paper. O.T. – clinical management and revising the paper. M.M. – critical contributions and revising the paper. A.G. – clinical management and revising the paper. A.R. – clinical management and revising the paper. P.M.S. – clinical management and revising the paper.


Supplementary Material



Publication History

Received: 29 August 2022

Accepted: 02 January 2023

Accepted Manuscript online:
05 January 2023

Article published online:
23 February 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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