Sezary Syndrome: Clinicopathological and Immunophenotypical Characteristics and Their Impact On Survival

Sezary syndrome is a rare leukemic subtype of cutaneous T cell lymphoma with aggressive clinical behavior and poor prognosis compare to the most common type of CTCL, mycosis fungoides. Hematologic criteria for the diagnosis of Sezary syndrome include an absolute Sezary cell count of >1000cells/mm³ in peripheral blood the presence of a clonal T cell population, increased CD4/CD8 ratio, and aberrant expression of T cell markers.

To evaluate clinical, pathological and immunophenotypical characteristic in patients with Sezary syndrome and determine the impact of these factors on disease survival.

Retrospective chart review of 17 consecutive patients diagnosed with Sezary syndrome at Moffitt Cancer Center from January 1998-June 2012. Data points collected were age, gender, stage of disease, date of diagnosis, date of death, and analysis of peripheral blood by flow cytometry. Statistical analyses were performed by SPSS statistical software (IBM, Somers, NY). All causes of death were included in the survival analysis. Survival curves were estimated by the Kaplan-Meier method.

Of the 17 patients evaluated 10 (58. 8%) were female, and 7 (41. 2%) were male, median age was 71 years (range 50–89). Patients were staged according to 2007 ISCL/EORTC staging system 8 (47. 1%) stage III and 9 (52. 9%) stage IV. Median survival was 20. 9 months (range 0–107. 6) for all stages; 27 months and 9. 36 months for stage III and IV, respectively. As expected, stage IV disease was associated with a higher CD4:CD8 ratio (median 62. 5/uL versus 8. 6/uL), absolute lymphocyte count (median 9, 860/ul versus 2800), and absolute Sezary cell count (median of 8, 700/uL versus 1, 700/uL). Interestingly, stage IV disease was also associated with a higher absolute NK cell count (median 280/uL versus 60/uL) and a lower absolute CD8 T cell count (median 165/uL versus 290/uL). Of all parameters studied the only ones showing statistical correlation with the stage of disease were the NK cell count (p = 0. 018) and the CD4:CD8 ratio (p = 0. 039). The immunophenotype of the Sezary cells did not correlate with stage of disease. Survival analysis did not show any significant differences by grouping patients according to high or low levels of variables described above, although the series is small.

We concluded that increased burden of disease in the peripheral blood did not affect overall survival in our patient population. However, Higher CD4:CD8 ratio, higher absolute lymphocyte count, and lower absolute CD8+ T cell count was associated with more advanced stage of disease suggesting that a lack of cytotoxic T cells can be responsible for profound immunosuppression and disease progression found in patients with advanced stage of CTCL.

No relevant conflicts of interest to declare.