What is the treatment of choice for von Willebrand disease (vWD) type 3?

Updated: Dec 30, 2019
  • Author: Eleanor S Pollak, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Answer

The treatment of choice for patients with vWD type 3 (as with other vWD types unresponsive to DDAVP) is rVWF (with or without FVIII) [24]  or virus-inactivated, vWF-containing FVIII concentrates that contain a near-normal complement of high-molecular-weight vWF multimers. [29] Most experience reported in the literature has been with the use of Humate-P, a plasma-derived FVIII/FVIII  of intermediate purity. Two other FVIII concentrates, Alphanate and Koate-HP, have been reported to be effective in the treatment of vWD. Too little vWF is present in monoclonally purified FVIII concentrates and recombinant FVIII concentrates to allow their use in the treatment of vWD.

Products that contain vWF only have been shown to correct not only the primary vWF deficiency but also the secondary FVIII:C deficiency, and have demonstrated safety and efficacy in various clinical situations, including surgery. However, their value for use in children, for long-term, prophylaxis, and in recurrent gastrointestinal bleeding due to angiodysplasia has yet to be explored. [30]


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