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Predictors of Response in Patients with Sézary Syndrome

March 2011

New Orleans—In a retrospective cohort study of 97 patients with Sézary syndrome receiving multiple immune-modifying therapies, the following factors were strongest for predicting complete response to treatment compared with no response: lower cluster of differentiation 4:cluster of differentiation 8 (CD4:CD8) ratio, higher percentage of monocytes, and lower numbers of circulating abnormal T cells at baseline. The results were presented at the AAD meeting during a poster session titled Clinical Response Rate of Sézary Syndrome with Immune Modifying Therapies: Prognostic Markers of Response. The study’s authors were from the University of Pennsylvania’s Department of Dermatology in Philadelphia. Patients with Sézary syndrome, the leukemic form of cutaneous T-cell lymphoma, have a median survival time between 2.5 and 5.0 years, and a 5-year survival rate of 30%. There are multiple treatment options available to treat Sézary syndrome, with the authors noting that extracorporeal photopheresis (ECP) is the first-line treatment for patients with erythrodermic psoriasis. The authors examined a database of 143 patients at the University of Pennsylvania who had received ECP from 1985 through 2010 to identify the prognostic parameters that affected response to ECP treatment. Previous studies have indicated there are various prognostic indicators, including age, steroid use, stage, lactate dehydrogenase, white blood cell count, extracutaneous disease, and beta-2 microglobulin. The authors selected 97 patients from the database who had stage 3A through stage 4B Sézary syndrome and received >2 months of ECP as well as ≥1 other treatment, such as interferon alpha, interferon gamma, sargramostim, or systemic retinoid. The database included >50 variables, including patient characteristics, laboratory values, and response results. Of the 97 patients, 73 (75.3%) showed significant improvement with the multimodality therapy; 29 (29.9%) had complete response, which was defined as complete resolution of the disease, and 44 (45.4%) achieved a partial response, which was defined as a ≥50% but <100% improvement. Compared with the group with no response, patients who had complete response had a lower CD4:CD8 ratio (13.2 vs 44.2; P=.04), median percentage of CD4+/CD26− cells (27.4% vs 57.2%; P=.01), and median percentage of CD4+/CD7− cells (20.1% vs 41.3%; P=.01). There were no statistically significant variables between the groups with partial response and no response. The authors concluded that if healthcare professionals gain a better understanding of predictive factors, they can achieve better outcome predictions for patients, determine the most effective multimodality treatment combination, and tailor the therapy to the disease’s aggressiveness. They also said it is important that they gain a further understanding of baseline monocyte levels.