With the introduction of multiple new effective therapeutic options for the treatment of macroglobulinemia, a structured approach to management of this rare lymphoma is currently needed. A review of phase II and III treatment trials over the past 20 years was performed, and high-quality trials are summarized in this manuscript. Because of the lack of large prospective trials comparing different classes of therapy, a uniform recommendation applicable to all patients cannot be made, and the approach must be individualized incorporating patient preferences, comorbidities, and the range of therapeutic toxicities. Therapeutic options for patients with newly diagnosed and previously treated macroglobulinemia are presented on the basis of the best available evidence in the literature.
ASJC Scopus subject areas
- Cancer Research