SYSTEMIC THERAPY OF CUTANEOUS T-CELL LYMPHOMAS (MYCOSIS-FUNGOIDES ANDTHE SEZARY-SYNDROME)

Objective: To review recent studies of systemic therapy for mycosis fu ngoides and the Sezary syndrome (cutaneous T-cell lymphomas). Data Sou rces: English-language articles indexed in MEDLINE from 1988 through 1 994. Study Selection: All therapeutic studies were selected. Data Extr action: The data were abstracted without judgments on response criteri a or patient numbers. Data quality and validity were assessed by indep endent author reviews. Data Synthesis: No systemic therapy cures patie nts with cutaneous T-cell lymphomas. Single and combined chemotherapeu tic agents produce high response rates. Whether any of these is prefer red is not established. A randomized trial comparing combination chemo therapy plus radiation therapy with topical therapy showed no survival benefit for the combination. Several adenosine analogs and retinoids were active, but their optimal use is uncertain. Interferons are as ac tive as chemotherapeutic agents and may be less toxic. Interferon comb ined with psoralen plus ultraviolet A light therapy produces high comp lete response rates and long-lasting remissions. Combinations with oth er systemic therapies do not increase response rates. Photopheresis th erapy should be regarded as experimental. Promising preliminary result s were seen with interleukin-2 fusion toxins and several antibody conj ugates. Conclusions: Systemic therapy should be considered effective a nd palliative. The principles of treating all low-grade lymphomas can be applied. Randomized trials are needed to evaluate new agents (such as a comparison of psoralen plus ultraviolet light with or without int erferon), and large phase II trials are needed for new agents such as photopheresis, interleukin-2 fusion toxin, temozolomide, and others.