Acquired von Willebrand Syndrome Associated to Secondary IgM MGUS Emerging after Autologous Stem Cell Transplantation for AL Amyloidosis

Mediterranean Journal of Hematology and Infectious Diseases
Hina QamarVictor H Jimenez-Zepeda

Abstract

Acquired 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.

Citations

Dec 12, 2017·Case Reports in Oncological Medicine·Sijan BasnetDilli Ram Poudel
Apr 28, 2020·Acta Haematologica·Iuliana Vaxman, Morie Gertz

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Methods Mentioned

BETA
urine collection
biopsy
enzyme-linked immunosorbent assay

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