Table 1.

Suggested Treatment Options for Waldenstrom Mcroglobulinemia

Asymptomatic patients

Observation alone

First-line therapy

Patients requiring rapid disease control Rituximab based combinations* Alkylating agents (eg, DRC, R-CHOP) Purine analogs (eg, FR) Bortezomib-based combinations (eg, BDR) Patients not requiring rapid disease control Single agent therapy (eg, rituximab, chlorambucil, fludarabine) Combination therapy Alkylating agent-based (eg, RCD, CVP-R, CP-R)† Nucleoside analog-based (eg, FR)*

Salvage therapy Reuse of first-line agent (for patients relapsing ≥2 years following initial treatment) Alternate first-line agents (for patients relapsing <2 years after initial therapy) Combination therapy as above Bortezomib-based therapy Immunomodulatory drugs Alemtuzumab Bendamustine Stem cell transplantation

BDR=bortezomib, dexamethasone, rituximab; CPR= cyclophosphamide, prednisone, rituximab; CVP-R= cyclophosphamide, vincristine, prednisone; DRC= dexamethasone, rituximab, cytoxan; FR=fludarabine, rituximab; R-CHOP=rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone

^*Transient flares in IgM levels have been reported in nearly half the patients treated with rituximab monotherapy.

^† For patients potentially eligible for autologous transplant, prolonged exposure to alkylating agents and nucleoside analogs should be limited prior to stem cell collection.