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Bortezomib-based Chemotherapy Regimen Well-tolerated in Waldenström Macroglobulinemia Associated With Acquired Von Willebrand Syndrome

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In patients with Waldenström macroglobulinemia (WM) with acquired von Willebrand syndrome (AVWS) the use of a bortezomib-based chemotherapy regimen is well tolerated and effective in long-term follow-up, according to an article in the Journal of Blood Medicine.

In this patient population treatment strategy includes rapidly treating emergency bleeding event and managing the underlying malignant lymphoid disorder, but there is no recommended standard of care.

In this report, 3 elderly patients were treated with a bortezomib-based chemotherapy regimen for several years before switching to the phosphatidylinositol 3-kinase isoform-delta inhibitor oral therapy, idelalisib.

This regimen was well tolerated. Adverse effects included changes in hepatic levels that were reversible.

The authors concluded that bortezomib-based therapy should be considered in refractory patients with AVWS associated with WM.



Ojeda-Uribe M, Rimelen V, Marzullo C. Good Profile of Efficacy/Tolerance of Bortezomib or Idelalisib in Waldenström Macroglobulinemia Associated with Acquired Von Willebrand Syndrome. J Blood Med. 2020;11:67‐72. Published 2020 Feb 13. doi:10.2147/JBM.S233059