What is the treatment approach for Waldenström macroglobulinemia?

Updated: Feb 01, 2021
  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Except for patients requiring emergency treatment of hyperviscosity syndrome, most patients can be treated as outpatients. Periodic physical examinations for organomegaly, routine chemistry evaluations, serum paraprotein level, serum viscosity, and coagulation tests should be performed to monitor for progression and to aid in treatment decisions. Patients requiring emergency plasmapheresis should be transferred to a center that offers this therapy.

For relapsed disease, patients who achieved a durable response (3 years or longer) with initial treatment and tolerated it well can be retreated with the original regimen. The US Food and Drug Administration (FDA) has approved an expanded indication for ibrutinib in WM beyond its use as a monotherapy to include combination use with rituximab. [30] In patients with relapsed or refractory disease harboring the MYD88 L265P mutation, ibrutinib is highly effective. A bortezomib-rituximab–based option is reasonable for patients with relapsed or refractory disease who do not have neuropathy.

Autologous stem cell transplantation should be considered at the first or second relapse in select patients with chemosensitive disease. Everolimus and purine analogs are suitable options for refractory or multiply relapsed WM. [4]

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