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Transition of Sézary syndrome into mycosis fungoides after complete clinical and molecular remission under extracorporeal photophoresis

¹ Department of Dermatology, Charité-Universitätsmedizin Berlin, Campus Benjamin Franklin, Fabeckstrasse 60-62, 14195 Berlin, Germany
² Institute of Pathology, Charité-Universitätsmedizin Berlin
³ Department of Neonatology and Neuropediatrics, Philipps University, Deutschhausstr. 12, 35037 Marburg, Germany

Correspondence to:
Dr C Assaf
Department of Dermatology, Charité-Universitätsmedizin Berlin, Campus Benjamin Franklin, Fabeckstrasse 60-62, 14195 Berlin, Germany; chalid.assaf{at}charite.de
ABSTRACT
Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common clinical variants of cutaneous T cell lymphoma. Although thought to be closely related to mature T helper cells, the relation between the neoplastic cells in MF and SS is still not fully clarified. This report describes a patient with complete remission of SS under treatment with extracorporeal photophoresis (ECP), who subsequently developed typical plaques of MF and large cell lymphoma (LCL). Serial polymerase chain reaction analyses confirmed identical T cell receptor ß and gene rearrangements in SS, MF, and LCL, and complete disappearance of the circulating malignant T cell clone from the peripheral blood after ECP. These findings indicate that the neoplastic cells in SS, MF, and LCL are derived from a common precursor T cell, despite the change in clinical phenotype.

Abbreviations: CTCL, cutaneous T cell lymphoma; ECP, extracorporeal photophoresis; LCL, large cell lymphoma; MF, mycosis fungoides; PCR, polymerase chain reaction; SS, Sézary syndrome; TCR, T cell receptor; Th, T helper cell

Keywords: cutaneous T cell lymphoma; mycosis fungoides; Sézary syndrome; T cell; receptor rearrangement; extracorporeal photophoresis