Salvage therapeutic options for Waldenström macroglobulinemia
Therapeutic class and agents . | Evidence for efficacy21 . | Level of recommendation21 . |
|---|---|---|
| Alkylator agents*† | ||
| Chlorambucil | IIa | B |
| Nucleoside analogs*† | ||
| Cladribine or fludarabine | Ib | A |
| Monoclonal antibody† | ||
| Rituximab (standard or extended schedule) | IIa | B |
| Alemtuzumab | III | C |
| Nucleoside analogs plus alkylators*† | ||
| Cladribine or fludarabine plus cyclophosphamide | IIa | B |
| Nucleoside analogs plus rituximab*† | ||
| Fludarabine plus rituximab | IIa | B |
| Nucleoside analogs plus alkylators and rituximab*† | ||
| Cladribine, cyclophosphamide, and rituximab | IIb | B |
| Fludarabine, cyclophosphamide, and rituximab | III | C |
| Pentostatin, cyclophosphamide, and rituximab | III | C |
| Combination chemotherapy plus rituximab | ||
| CHOP and rituximab | III | C |
| Thalidomide | ||
| Thalidomide alone or in combination with dexamethasone | IIa | B |
| Stem cell transplantation‡ | ||
| High-dose chemotherapy and autologous stem cell transplantation | IIa | B |
Therapeutic class and agents . | Evidence for efficacy21 . | Level of recommendation21 . |
|---|---|---|
| Alkylator agents*† | ||
| Chlorambucil | IIa | B |
| Nucleoside analogs*† | ||
| Cladribine or fludarabine | Ib | A |
| Monoclonal antibody† | ||
| Rituximab (standard or extended schedule) | IIa | B |
| Alemtuzumab | III | C |
| Nucleoside analogs plus alkylators*† | ||
| Cladribine or fludarabine plus cyclophosphamide | IIa | B |
| Nucleoside analogs plus rituximab*† | ||
| Fludarabine plus rituximab | IIa | B |
| Nucleoside analogs plus alkylators and rituximab*† | ||
| Cladribine, cyclophosphamide, and rituximab | IIb | B |
| Fludarabine, cyclophosphamide, and rituximab | III | C |
| Pentostatin, cyclophosphamide, and rituximab | III | C |
| Combination chemotherapy plus rituximab | ||
| CHOP and rituximab | III | C |
| Thalidomide | ||
| Thalidomide alone or in combination with dexamethasone | IIa | B |
| Stem cell transplantation‡ | ||
| High-dose chemotherapy and autologous stem cell transplantation | IIa | B |
Information has been updated from the original consensus panel recommendations of the Second International Workshop on Waldenström's Macroglobulinemia.3
The choice of appropriate therapy should take into account the candidacy of a patient for high-dose chemotherapy since prolonged use of both alkylating agents and nucleoside analogs can deplete hematopoietic stem cells.
Reuse of a frontline single agent or combination is reasonable if patient achieved a response duration of at least 1 year; otherwise, use of an alternate single agent or combination is reasonable.
For eligible patients with primary refractory or relapsed disease, high-dose chemotherapy with autologous stem cell transplantation may be reasonable; allogeneic or “nonmyeloablative allogeneic” transplantation procedures should be approached cautiously in view of the associated high mortality and/or morbidity risks and should be undertaken in context of a clinical trial.