What are the treatment recommendations from the International Workshops on Waldenström macroglobulinemia?

Updated: Feb 01, 2021
  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Treatment recommendations from the 10th International Workshop on Waldenström Macroglobulinemia are as follows [31] :

  • Alkylating drugs (bendamustine, cyclophosphamide) and proteasome inhibitors (bortezomib, carfilzomib, ixazomib), both in combination with rituximab, as well as Bruton’s tyrosine kinase (BTK) inhibitors (ibrutinib), alone or in combination with rituximab, are preferred first-line therapy options for symptomatic patients. MYD88 and L265P mutation testing should be performed before selection of ibrutinib.

  • In previously treated patients with who had an initial durable response, reuse of a previous regimen or another primary therapy regimen are acceptable options.

  • Novel BTK inhibitors (acalabrutinib, zanubrutinib, tirabrutinib) and the BCL2 antagonist venetoclax appear safe and active, and represent emerging treatment options.

  • The choice of therapy should be guided by the patient’s clinical profile, genomic features, and drug availability

  • Autologous stem cell transplantation may be considered following second or subsequent relapses despite treatment with chemoimmunotherapy and BTK inhibitors, in high-risk patients with chemosensitive disease.

In addition, clinical trials are currently exploring the following for treatment of WM [31] :

  • Proteasome inhibitors - Ixazomib, dexamethasone, and rituximab
  • BTK inhibitors - Zanubrutinib, tirabrutinib; second generation, non-covalent BTK inhibitors (eg, vecabrutinib, LOXO-305) are being investigated for use in WM refractory to ibrutinib
  • The anti-CD38 monoclonal antibody  daratumumab [32]

See also Waldenstrom Macroglobulinemia Treatment Protocols.

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