Table 1.
Recommended Strategy for the Treatment of ATL
| Smoldering- or favorable chronic-type ATL |
| Consider inclusion in prospective clinical trials |
| Symptomatic patients (skin lesions, opportunistic infections, and so on): consider AZT/IFN-α or watch and wait |
| Asymptomatic patients: consider watch and wait |
| Unfavorable chronic- or acute-type ATL |
| Recommend: inclusion in prospective clinical trials |
| If outside clinical trials, check prognostic factors (including clinical and molecular factors if possible): |
| Good prognostic factors: consider chemotherapy (VCAP-AMP-VECP evaluated by a randomized phase III trial against biweekly CHOP) or AZT/IFN-α (evaluated by a retrospective worldwide meta-analysis) |
| Poor prognostic factors: consider chemotherapy followed by conventional or reduced-intensity allogeneic HSCT (evaluated by retrospective or prospective Japanese analyses, respectively) |
| Poor response to initial therapy with chemotherapy or AZT/IFN-α: consider conventional or reduced-intensity allogeneic HSCT |
| Lymphoma-type ATL |
| Recommend: inclusion in prospective clinical trials |
| If outside clinical trials, consider chemotherapy (VCAP-AMP-VECP) |
| Check prognostic factors and response to chemotherapy (including clinical and molecular factors if possible): |
| Favorable prognostic profiles and good response to initial therapy: consider chemotherapy |
| Unfavorable prognostic profiles or poor response to initial therapy with chemotherapy: consider conventional or reduced-intensity allogeneic HSCT |
| Options for clinical trials (first line) |
| Test the effect of up-front allogeneic HSCT |
| Test promising targeted therapies such as arsenic trioxide + IFN-α, bortezomib + chemotherapy, or antiangiogenic therapy |
| Consider a phase II global study testing pegylated IFN and AZT |
| Options for clinical trials (relapse or progressive disease) |
| Test the effect of promising targeted therapies such as arsenic trioxide and IFN-α, bortezomib, a purine nucleotide phosphorylase inhibitor, histone deacetylase inhibitors, monoclonal antibodies, antiangiogenic therapy, and survivin, β-catenin, syk, and lyn inhibitors, etc. |
| Consider conventional or reduced-intensity allogeneic HSCT when possible |
Abbreviations: ATL, adult T-cell leukemia-lymphoma; AZT, zidovudine; IFN-α, interferon alfa; VCAP-AMP-VECP, vincristine, cyclophosphamide, doxorubicin, and prednisone; doxorubicin, ranimustine, and prednisone; and vindesine, etoposide, carboplatin, and prednisone; CHOP, cyclophosphamide, doxorubicin, vincristine, and prednisone; HSCT, hematopoietic stem-cell transplantation.