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Use of Vonicog Alpha and Acquired von Willebrand Syndrome, a New Approach: A Case Report.
Blandinières, Adeline; Combe, Sophie; Chanson, Noémie; Lambotte, Olivier; Lavenu-Bombled, Cécile.
Afiliación
  • Blandinières A; Department of Hematology, Public Assistance Hospitals of Paris (AP-HP), Bicêtre Hospital, Le Kremlin-Bicêtre, France.
  • Combe S; University of Paris-Saclay, INSERM Hemostasis, Inflammation and Thrombosis Unit (HITh) U1176, Le Kremlin-Bicêtre, France.
  • Chanson N; Department of Hematology, Public Assistance Hospitals of Paris (AP-HP), Bicêtre Hospital, Le Kremlin-Bicêtre, France.
  • Lambotte O; Department of Internal Medicine and Clinical Immunology, Public Assistance Hospitals of Paris (AP-HP), Bicêtre Hospital, Le Kremlin-Bicêtre, France.
  • Lavenu-Bombled C; Department of Internal Medicine and Clinical Immunology, Public Assistance Hospitals of Paris (AP-HP), Bicêtre Hospital, Le Kremlin-Bicêtre, France.
Hamostaseologie ; 2024 Mar 22.
Article en En | MEDLINE | ID: mdl-38519044
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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Hamostaseologie Año: 2024 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Alemania
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Hamostaseologie Año: 2024 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Alemania