1 Department of Medicine, University of Alberta, Edmonton, AB, Canada.
2 Southern Alberta Rare Blood & Bleeding Disorders Comprehensive Care Program, Department of Medicine, Calgary, AB, Canada.
3 Tom Baker Cancer Center, Department of Medical Oncology and Hematology, Calgary, AB, Canada.
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von Willebrand syndrome (AVWS) is a rare hemorrhagic disorder that
occurs in patients with no prior personal or family history of
bleeding. Here, we describe a case of AVWS occurring after autologous
stem cell transplantation (ASCT). Interestingly, AVWS developed after
bortezomib-based induction and conditioning regimens. Recent evidence
suggests that the proximity of the bortezomib therapy to the collection
of stem cells with consequent depletion of regulatory T cells after the
conditioning regimen could explain some of the unusual autoimmune
complications reported in patients receiving bortezomib prior to ASCT.
In addition, this patient developed a secondary MGUS post-ASCT, which
may have also contributed to the AVWS. To the best of our knowledge,
this is the first case of post-ASCT AVWS reported. Prospective data is
needed to better elucidate the mechanisms by which these unusual
complications occur in patients receiving bortezomib prior to ASCT.
|Table 1. VWF, VWFpp, and VWFpp:Ag at diagnosis, during DDAVP testing, and in remission.|
|Figure 1. Levels of von Willebrand factor VIII Ag and Ristocetin Cofactor activity. (In the X axis, days from episode of bleeding is shown, while in the Y axis levels in U/mL are recorded).|
|Figure 2. Multimer gel image demonstrated the presence of high molecular weight multimers.|