Treatment of Sezary Syndrome with Combined Therapy

Abstract 4928

The Sezary syndrome (SS) is an erythrodermic cutaneous T-cell lymphoma with a leukemic component and lymphadenopathy, characterized by atypical T-lymphocytes, and a CD4 + T-helper/memory; these cells may express the markers CD45RO, and CD25 with frequent loss of the CD26, CD7, CD2, CD3, and with cerebriform nuclei.

The strategies for patients with SS in early stage, when the disease is limited to the skin, includes topical therapies with corticosteroids (clobetasol), carmustine, retinoids (bexarotene, tazarotene) and phototherapy with PUVA (psoralen and ultraviolet light), in the refractory or relapsed patients polychemotherapies (CMED/AB, Pegylated liposomal doxorubicin, gemcitabine) may reduce tumor burden, as well as the symptoms, and most interestingly, it may delay tumor progression and preserve significantly the quality of life; the most typical approach might be constituted by a high dose of chemotherapy followed by allogenic bone marrow transplantation.

We have studied 2 patients with advanced stage IIB. One of them was a 52-year old patient (T3, NP0, M0, B0), and the other one was a 62-year old patient (T3, NP1, M0, B1); both of them were treated with the following drug-combination: bexarotene 150 mg os daily, and vorinostat 400 mg os once daily, and localized treatment with clobetasol. This scheme had a significantly high clinical response and little side effects (fatigue, lethargy, thrombocytopenia, headache), and the patients remained in remission the first for 4 years DFS and the latter for 3 years DFS; both of them were initially treated with PUVA, MTX 50 mg, and INF-alfa 3 MU per day.