Which of the following is the best treatment for a hemodialysis patient with severe bleeding and type I von Willebrand disease?
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Type I von Willebrand disease (vWD) is caused by decreased production of von Willebrand factor. vWF is produced and stored in both platelets and vascular endothelial cells, and DDAVP (or desmopressin) can stimulate the release of available von Willebrand factor in most patients to increase the serum levels and assist in clot formation. A side effects of DDAVP is hyponatremia so serum sodium levels should be monitored in patients receiving frequent DDAVP doses to control bleeding.
Fresh frozen plasma contains von Willebrand factor in it and would be effective therapy for severe bleeding however the large volume of infusion would make it a poor choice for a patient on hemodialysis who can get fluid overloaded easily. Cryoprecipitate, however, contains large amounts of von Willebrand factor and can be used for replacement. Endogenous von Willebrand factor in the bloodstream is bound to factor VIII and protects factor III from degradation, and so the best treatment option for bleeding patients with reduced von Willebrand factor or for those who do not respond to DDAVP is replacement with partially purified factor VIII products, which also contain large amounts of von Willebrand factor.
Recombinant factor VIII products are composed solely of purified factor VIII and do not contain any von Willebrand factor.
Except in cases of type IIB vWD that are associated with thrombocytopenia, platelet counts are generally normal in vWD patients, and so transfusions would not be of any benefit to control bleeding.
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