Long-Term Complete Responses to Combination Therapies in Sézary Patients.

Sézary syndrome (SS), the leukemic variant of cutaneous T-cell lymphoma (CTCL), is defined as erythroderma and >1000 abnormal T-cells per mm³ in the peripheral blood, and is currently incurable. A recent study of erythrodermic CTCL conducted at our center reported a median overall survival of 5.1 years which is twice that in the literature [Vidulich K, et al. Int. J of Dermatol. 2009; 48: 243-252]. Although complete remissions (CR) may be achieved in earlier stages of CTCL, they are rarely achieved in SS patients.

A retrospective analysis of a prospective database of 1550 CTCL patients seen at the MD Anderson Cancer Center since 1984 identified a subset of 145 SS patients (9%) whose long-term complete responses (>2 years) to various therapies is reported. The most commonly used combination therapies were topical steroids, psoralen with ultraviolet A (PUVA), oral retinoids (isotretinoin, acetretin, or bexarotene), alpha-interferon, and extracorporeal photopheresis (ECP). A combination of total skin electron beam radiation (TSEB) followed by induction chemotherapy and allogeneic stem-cell transplant was given to 23 CTCL patients. Chronic Staphylococcus aureus carriers identified by cultures were treated with antibiotics and preventive skin care.

Of the 145 Sézary Syndrome patients, 13 (8.96%) achieved CRs, including 6 females and 7 males. Their median age was 48 (20 to 85 years) at diagnosis. Nine were Caucasian, 3 were African American, and 1 was Hispanic. At initial diagnosis their stages were IB (n=1), stage III (n=3), stage IVA (n=4), and stage IVB (n=5). Six achieved a CR on front-line combination immunomodulatory therapy consisting of ECP with alpha-interferon and/or oral retinoids, antibiotics, and topical steroids. Seven SS patients failed therapy but were able to obtain CRs after TSEB and allogeneic peripheral blood stem cell transplantation (SCT) from matched related donors (n=6) or unrelated (n=1) donors. The conditioning regimen for SCT was fludarabine and melphalan. The median time between diagnosis and CR was 4.07 years (range 1 to 20 years). The median duration of CR is 5 years (mean 5.15 years, range 2 to 13 years).

Sézary Syndrome is extremely symptomatic and usually refractory to current therapies. CRs may be achieved using immunomodulatory therapy with biological response modifiers and photopheresis or with total skin electron beam followed by non-ablative allogeneic stem cell transplant in appropriate patients and may have an impact on overall survival.

No relevant conflicts of interest to declare.