Strategy for the treatment of Adult T-Cell Leukemia-Lymphoma.
Smoldering- or favorable chronic-type ATL
(i) Consider inclusion in prospective clinical trials.
(ii) Symptomatic patients (skin lesions, opportunistic infections, etc.): Consider AZT/IFN or Watch and Wait.
(iii) Asymptomatic patients: Consider Watch and Wait.
Unfavorable chronic- or acute-type ATL
(i) If outside clinical trials, check prognostic factors (including clinical and molecular factors if possible):
(a) Good prognostic factors: consider chemotherapy (VCAP-AMP-VECP evaluated by a phase III trial against biweekly-CHOP) or AZT/IFN (evaluated by a meta-analysis on retrospective studies).
(b) Poor prognostic factors: consider chemotherapy followed by conventional or reduced intensity allo-HSCT (evaluated by retrospective and prospective Japanese analyses, resp.).
(c) Poor response to initial therapy: Consider conventional or reduced intensity allo-HSCT.
Lymphoma-type ATL
(i) If outside clinical trials, consider chemotherapy (VCAP-AMP-VECP).
(ii) Check prognostic factors (including clinical and molecular factors if possible) and response to chemotherapy:
(a) Good prognostic factors and good response to initial therapy: Consider chemotherapy followed by observation.
(b) Poor prognostic factors or poor response to initial therapy: Consider chemotherapy followed by conventional or reduced intensity allo-HSCT.
