On the Way to Curing Advanced-Stage Mycosis Fungoides/Sézary Syndrome

• Published in: Clinical lymphoma, myeloma and leukemia
• Main Authors: Purnak, Seda, Hosing, Chitra, Dabaja, Bouthaina, Bassett, Roland L., Huen, Auris, Duvic, Madeleine
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 14.07.2024
• Subjects: Allogeneic stem cell transplant; Cutaneous T-cell lymphoma; Mycosis fungoides; Sézary syndrome; Total skin electron beam; B; CTCL; MRI; SCT; USCLC; GVHD; Total skin electron beam; FLU; IFN; BSA; PUVA; CMED; Allogeneic stem cell transplant; RTX; EPOCH; CHOP; MF; XRT; PFS; Cutaneous T-cell lymphoma; SS; OS; EORTC; Mycosis fungoides; IVIG; Sézary syndrome; CR; CT; ISCL; ECP; ATG; MEL; CY; MDACC; NB-UVB; TSEB; GVL; LCT; PET
• ISSN: 2152-2650; 2152-2669; 2152-2669
• DOI: 10.1016/j.clml.2024.07.011

Advanced-stage mycosis fungoides (MF) and Sézary syndrome (SS) have poor prognosis with median survivals of less than 5 years. Although a variety of treatments are approved for MF/SS patients, durable complete remissions (CR) are rare. Advanced-stage MF or SS patients who achieved CR and maintained in CR or stage IA for more than 10 years were identified by a retrospective search of the principal investigator's database. Of 2266 patients diagnosed with MF or SS, 23 patients with advanced-stage MF/SS (6 IIB, 1 IIIB, 5 IVA1, 3 IVA2, 8 IVB) who achieved CR and maintained in CR or stage IA for ≥ 10 years were identified. As final/curative treatment, 11 patients underwent allogeneic stem cell transplantation (SCT). Most patients presented at young age, underwent SCT with reduced intensity conditioning regimen, had matched related donors, and controllable post-transplant graft versus host disease. Eleven patients were treated with TSEB as part of combined modality protocol in 2 patients and debulking therapy before allogeneic SCT in 9 patients. Five stage IIB patients achieved CR with radiotherapy. Four patients with blood involvement were treated with extracorporeal photopheresis (ECP) in combination with long-term antibiotics and immunomodulatory agents. Long-term antibiotics were given to 14 patients. TSEB followed by allogeneic SCT, radiotherapy, ECP plus long-term antibiotics and immunomodulatory agents were the most common curative/final treatments found in our patients. We are reporting the details of our long-term complete responders’ treatment course in the hopes of obtaining more cure responses in the future. Advanced-stage mycosis fungoides (MF) and Sézary syndrome (SS) have poor prognosis. In this retrospective study of 2266 MF/ SS patients, 23 patients with advanced-stage MF/SS who achieved complete remission and relapsed no greater than stage IA for ≥ 10 years were identified. Total skin electron beam followed by allogeneic stem cell transplantation, radiotherapy, extracorporeal photopheresis plus long-term antibiotics and immunomodulatory agents were the most common curative/final treatments.