Figure 1

Plasma levels of factor VIII clotting activity (FVIII:C) and von Willebrand factor parameters (VWF:Ag and VWF:Ac) at presentation and during course of treatment. The first set of data points (d0) shows FVIII and VWF levels at initial presentation, while the second set of data points (d3) shows FVIII and VWF levels immediately before the administration of 2,000 IU of FVIII/VWF concentrate, as indicated by the dashed arrow. To assess incremental recovery and residence time of the infused FVIII/VWF, subsequent blood samples were drawn 15 min (d3´), 60 min (d3´´), and 120 min (d3´´´) after concentrate administration. One day later (d4), immunosuppressive therapy with prednisolone was started at a daily dose of 100 mg, which was gradually tapered to 20 mg on d31, as indicated by the solid arrows. One week later, there was a steep decline in FVIII and VWF, both of which readily responded to re-escalating the prednisolone dose to 50 mg per day.