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Acquired von Willebrand syndrome (AVWS) is a rare coagulation disorder that may be associated with IgM paraproteinemia
Vascular Recently, recombinant von Willebrand factor (rVWF) has been used to treat bleeding in patients with hereditary von Willebrand disease, but experience in patients with AVWS is limited
Patient 1 was an 80-year-old man with recurrent severe transurethral bleeding with a hemoglobin level of 6.
Table 1: Patient characteristics and laboratory results at presentation
Table 1: Patient characteristics and laboratory results at presentation Table 1: Patient characteristics and laboratory results at presentationFigure
Figure 1: Multimer analysis of patient plasma at presentation
But the transurethral bleeding did not resolve The transurethral bleeding stopped and did not recur
Immediately thereafter, immunosuppressive therapy with prednisolone and rituximab was started, but VWF activity did not improve and bleeding remained non-resolved
2
Patient 2 was a 71-year-old man who presented with epistaxis and severe intestinal bleeding after colon polyps removal, requiring transfusion of red blood cell concentrates
Patient 2 was a 71-year-old man who presented with epistaxis and severe intestinal bleeding after colon polyps removal, requiring transfusion of red blood cell concentrates
The research team believes that it is safe to use rVWF and VWF-specific IgM antibodies to fully increase VWF activity and stop bleeding
Original source:
Original source:Höpting M, Budde U, Tiede A, Grube M, Hahn J, Herr W, Heimerl S, Hart C.
Höpting M, Budde U, Tiede A, Grube M, Hahn J, Herr W, Heimerl S, Hart C.
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