Advantage of recombinant von Willebrand factor for peri‐operative management in paediatric acquired von Willebrand syndrome

Weyand, Angela C.; Jesudas, Rohith; Pipe, Steven W.

doi:10.1111/hae.13436

Cited by 7 publications

(2 citation statements)

References 10 publications

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“…Weyand et al reported the use of vonicog α in a child with AVWS associated with pulmonary valve stenosis who required cranioplasty following a skull fracture. 4 Höpting et al described the case of a patient with IgM-associated MGUS treated with recombinant factor Willebrand for acute bleeding episodes. 5 Recently, Heubner et al published the case of an adolescent undergoing ECMO in the context of COVID-19, who developed AVWS.…”

Section: Discussionmentioning

confidence: 99%

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

Blandinières,

Combe,

Chanson

et al. 2024

Hamostaseologie

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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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Section: Discussionmentioning

confidence: 99%

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

Blandinières,

Combe,

Chanson

et al. 2024

Hamostaseologie

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“…In this setting, experience with rVWF is very limited. So far, it has been successfully used in a patient with AVWS with an underlying cardiac disease [9]. It has been previously described that the administration of rVWF in patients with MGUS and smoldering myeloma was not effective [3].…”

Section: Discussionmentioning

confidence: 99%

Distinct Mechanisms of IgM Antibody-Mediated Acquired von Willebrand Syndrome and Successful Treatment with Recombinant von Willebrand Factor in One Patient

Höpting¹,

Budde²,

Tiede³

et al. 2022

Acta Haematol

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Acquired von Willebrand Syndrome (AVWS) is a rare coagulation disorder which can be associated with IgM-paraproteinaemia. Recently, recombinant von Willebrand factor (rVWF) has become available for the treatment of bleedings in patients with inherited von Willebrand disease, but experience in patients with AVWS is limited. We report on two patients with AVWS with underlying IgM paraproteinaemia with distinct underlying pathomechanisms. In one patient the paraprotein built unspecific complexes with von Willebrand factor. In the other patient, we were able to detect an IgM antibody against von Willebrand factor. Bleeding in this patient was successfully treated with rVWF. To our knowledge, this is the first report about the successful use of rVWF in a patient with AVWS with the detection of a von Willebrand factor-specific antibody.

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Vonicog alfa for the management of von Willebrand disease: a comprehensive review and single-center experience

Tran

Arnall

Moore

et al. 2020

J Thromb Thrombolysis

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Advantage of recombinant von Willebrand factor for peri‐operative management in paediatric acquired von Willebrand syndrome

Cited by 7 publications

References 10 publications

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

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

Distinct Mechanisms of IgM Antibody-Mediated Acquired von Willebrand Syndrome and Successful Treatment with Recombinant von Willebrand Factor in One Patient

Vonicog alfa for the management of von Willebrand disease: a comprehensive review and single-center experience

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