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Laboratory Assessment of Treatment of von Willebrand Disease
von Willebrand disease (VWD) may be treated by 1) raising the patient’s own von Willebrand factor (VWF), 2) replacement of VWF, or 3) use of adjuvant therapies with a global effect on hemostasis. A treatment plan must be based on an accurate diagnosis of the type and severity of VWD present in the patient, as described in Chapter 134 , and the specific clinical situation (e.g., active bleeding, minor surgical or dental procedure, or major surgery).
Methods
Laboratory tests used to monitor treatment of VWD, including VWF activity assays, factor VIII activity (VIII:C), and platelet function analyzer (PFA), are described in Chapter 134, Chapter 138 . VIII:C level is thought to be the most important determinant for surgical, soft tissue, and joint bleeding, whereas VWF level is more important for mucous membrane bleeding. Most reports describe use of ristocetin cofactor (VWF:RCo) for measuring VWF activity rather than the newer VWF activity assays discussed in Chapter 134 . Newer assays have advantages for monitoring treatment because they can be performed more rapidly. Although few comparisons in posttreatment specimens have been reported, results are expected to correlate with VWF:RCo. The PFA closure time (CT) has also been used to monitor therapy.
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