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Background: Sézary syndrome (SS) is a rare lymphoproliferative neoplasm, almost incurable outside the setting of allogeneic transplantable patients. Prognosis for relapse/refractory patient remains poor, as the available drugs confer short lasting remission. In this setting, the anti-chemokine receptor type 4 (CCR4) monoclonal antibody mogamulizumab demonstrated efficacy in an international, open label, randomized controlled phase 3 trial (MAVORIC) versus vorinostat.
Case description: A heavily pretreated 57-year-old SS woman (stage IVA) was randomized in the mogamulizumab arm of MAVORIC at our Institution. She quickly achieved a response but after 30 cycles she was discontinued from therapy due to cutaneous toxicity. Nevertheless, she is still in complete response (CR).
Conclusions: mogamulizumab is an anti-CCR4 monoclonal antibody that can induce long lasting response also in very heavily pre-treated patients not responding to any previous treatment. The extraordinary characteristic of our patient is that she is still in CR after 2.5 years since treatment discontinuation.