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 non-bulky 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 harbouring MYD88 L265P mutation
• in the absence of neuropathy, a bortezomib-rituximab-based option is reasonable for relapsed or refractory disease
• in select patients with chemo-sensitive disease, autologous stem cell transplantation should be considered at first or second relapse
• everolimus and purine analogues are suitable options for refractory or multiply relapsed disease

Reference:

1. Pratt G, El-Sharkawi D, Kothari J, et al. Diagnosis and management of Waldenström macroglobulinaemia - a British Society for Haematology guideline. Br J Haematol. 2022 Apr;197(2):171-87.
2. Kastritis E, Leblond V, Dimopoulos MA, et al. Waldenström's macroglobulinaemia: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018 Oct 1;29 (suppl 4):iv41-50.