Sezary Syndrome Aberrant Expression of CD26 On CD4+, CD25+, FOXP3+ T Lymphocytes and Prognostic Significance.

Abstract 4244

The Sezary syndrome (SS) is a variant of cutaneous lymphoma (CTLC) the cell is a malignant pleomorphic CD4+/CD25+/FOXP3+ T cell with a convolute-cerebriform nucleus, a cytogenetic evidence of an abnormal clone were observed in all patients MTAP (17p13.1-TP53), SMARCE (17q21, 1q21.3- STAT5A/STATB), DULLARD (9p21.3-CDKN2A/CDKN2B), M13930 (8q24.21-C-MYC/PLEC1), expression profiles showed upregulation of genes involved in TNF-signaling pathaway, and decreased expression of tumor suppressor genes such as TGF-beta receptor II, while TWIST and EPHA4; BCL-2, and TEX10 were overexpressed. Aberrant expression of GATA -3, TRAIL, T-plastin, CD1D are present in more advanced stages and correlate with disease progression. The most characteristic symptons include a persistent xerosis, erythematous patches, pruritus, ectropion, alopecia, dystrophic nails, palmoplantar keratoderma lymphadenopathy, hepatosplenomegaly, and sometime fungal infection or colonization with staphylococcus aureus. The loss of CD26,and a decreasing of CD4+CD7- expression,and an increasing of CD25+(alfa-chain of IL-2 receptor) FOXP3+ Tregs is a costant feature; the association with the circulating aneuploid cells showed high expression of skin manifestation, disease progression and poorest prognosis.