Treatment

Seek expert advice.

Suggested management (1,2):

• primary therapy for bulky disease, profound hematologic compromise, or constitutional symptoms
  • bendamustine-rituximab
  • dexamethasone-rituximab-cyclophosphamide is an alternative, particularly for nonbulky disease
• routine rituximab maintenance should be avoided
• plasma exchange
  • should be promptly initiated before cytoreduction for hyperviscosity-related symptoms
• stem cell harvest for future use may be considered in first remission for patients 70 years or younger who are potential candidates for autologous stem cell transplantation

Relapse management

• retreatment with the original therapy is reasonable in patients with prior durable responses (time to next therapy >=3 years) and good tolerability to previous regimen
• ibrutinib is efficacious in patients with relapsed or refractory disease harboring MYD88 L265P mutation
• in the absence of neuropathy, a bortezomib-rituximab-based option is reasonable for relapsed or refractory disease
• in select patients with chemosensitive disease, autologous stem cell transplantation should be considered at first or second relapse
• everolimus and purine analogs are suitable options for refractory or multiply relapsed disease

Reference:

• Kapoor P et al. Diagnosis and Management of Waldenström Macroglobulinemia - Mayo Stratification of Macroglobulinemia and Risk-Adapted Therapy (mSMART) Guidelines 2016.JAMA Oncol. 2017 Sep 1; 3(9): 1257-1265.
• Gertz MA. Waldenstrom macroglobulinemia: 2017 update on diagnosis, risk stratification, and management.Am J Haematol. 2017 Feb;92(2):209-217. doi: 10.1002/ajh.24557.