Good Profile of Efficacy/Tolerance of Bortezomib or Idelalisib in Waldenstrom Macroglobulinemia Associated with Acquired Von Willebrand Syndrome

Acquired von Willebrand syndrome (AVWS) in the setting of Waldenstrom macroglobulinemia (WM) is a challenging condition. No real standard of care is recommended for these patients, although the therapeutic strategy should include a rapid approach to the emergency bleeding events and to the underlyin...

Full description

Saved in:
Bibliographic Details
Published in:Journal of blood medicine 2020-02, p.67
Main Authors: Ojeda-Uribe, Mario, Rimelen, Valerie, Marzullo, Catherine
Format: Article
Language:English
Subjects:
Antineoplastic agents
Bortezomib
Elderly
Elderly patients
Health aspects
Ibrutinib
Idelalisib
Setting (Literature)
Vincristine
Von Willebrand factor
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Acquired von Willebrand syndrome (AVWS) in the setting of Waldenstrom macroglobulinemia (WM) is a challenging condition. No real standard of care is recommended for these patients, although the therapeutic strategy should include a rapid approach to the emergency bleeding events and to the underlying malignant lymphoid disorder. We report here our experience treating three elderly patients with these concomitant hematologic entities. The use of a bortezomib-based chemotherapy regimen showed a good profile of tolerance and efficacy even in a long-term follow-up period. These patients were treated for several years before switching their therapy to idelalisib, a targeted oral therapy that inhibits phosphatidylinositol 3-kinase isoform-delta (PI3KD), which is part of the signaling pathway downstream B-cell receptor. This approach was well tolerated and efficacious, although some adverse effects were observed, particularly at hepatic levels, but were all reversible. The same profile of tolerance/efficacy was observed in one very old patient who received idelalisib as a first-line therapy. We think that bortezomib-based therapy could be considered in refractory patients with AVWS associated with WM. Keywords: waldenstrom macroglobulinemia, acquired von Willebrand syndrome, bortezomib, idelalisib, proteasome inhibitors, von Willebrand factor
ISSN:1179-2736
1179-2736
DOI:10.2l47/JBM.S233059