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Hamostaseologie
DOI: 10.1055/a-2266-7984
Case Report

Use of Vonicog Alpha and Acquired von Willebrand Syndrome, a New Approach: A Case Report

Adeline Blandinières
1   Department of Hematology, Public Assistance Hospitals of Paris (AP-HP), Bicêtre Hospital, Le Kremlin-Bicêtre, France
2   University of Paris-Saclay, INSERM Hemostasis, Inflammation and Thrombosis Unit (HITh) U1176, Le Kremlin-Bicêtre, France
,
Sophie Combe
1   Department of Hematology, Public Assistance Hospitals of Paris (AP-HP), Bicêtre Hospital, Le Kremlin-Bicêtre, France
,
Noémie Chanson
3   Department of Internal Medicine and Clinical Immunology, Public Assistance Hospitals of Paris (AP-HP), Bicêtre Hospital, Le Kremlin-Bicêtre, France
,
Olivier Lambotte
3   Department of Internal Medicine and Clinical Immunology, Public Assistance Hospitals of Paris (AP-HP), Bicêtre Hospital, Le Kremlin-Bicêtre, France
4   University of Paris-Saclay, INSERM, CEA, UMR 1184, Le Kremlin-Bicêtre, France
,
Cécile Lavenu-Bombled
1   Department of Hematology, Public Assistance Hospitals of Paris (AP-HP), Bicêtre Hospital, Le Kremlin-Bicêtre, France
2   University of Paris-Saclay, INSERM Hemostasis, Inflammation and Thrombosis Unit (HITh) U1176, Le Kremlin-Bicêtre, France
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Abstract

Therapeutic management of acquired von Willebrand syndrome (AVWS) can be challenging, particularly in cases of AVWS associated with monoclonal IgM such as Waldenström macroglobulinemia (WM) where several therapeutic options may be ineffective. Here, we describe the case of an 88-year-old patient who developed AVWS during follow-up for WM. The presence of a severe bleeding symptomatology not controlled by several therapies (plasma-derived von Willebrand factor, plasmapheresis) led us to introduce a supplementation with recombinant von Willebrand factor, vonicog α (Veyvondi, Takeda, Japan), starting at a dose of 50 IU/kg/d. This supplementation allowed clinical (no further bleeding) and biological (hemoglobin level, von Willebrand factor parameters) improvements. Because of the persistence of bleeding risk factors, the treatment was maintained at a prophylactic dose (20 UI/kg three times a week), without recurrence of bleeding events for a period of 9 months.

Keywords

von Willebrand disease - Waldenström macroglobulinemia - prophylaxis - von Willebrand factor

Authors' Contributions

A.B. and C.L-B. drafted the case report. All authors reviewed and approved the final manuscript for publication.




Publication History

Received: 06 September 2023

Accepted: 11 February 2024

Article published online:
22 March 2024

© 2024. Thieme. All rights reserved.

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