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Optimizing CTCL Care: Expert Perspectives on Patient-Centered Approaches - Episode 11

Personalizing Care: Treatment Decisions in Sézary Syndrome

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Panelists discuss how to sequence and personalize therapy for Sézary syndrome, with mogamulizumab often used as first-line treatment due to potential for durable remission, followed by HDAC inhibitors, photopheresis, or alemtuzumab depending on disease progression and transplant planning.

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    Dr. Pinter-Brown discusses his preferred first-line treatment for Sézary syndrome using off-label mogamulizumab, based on promising results from the MAVORIC trial (NCT01728805). The treatment approach focuses on achieving complete response (CR) rates, with patients potentially discontinuing therapy after 12 months if they achieve CR. This time-limited treatment strategy represents an unusual approach for mycosis fungoides, as most treatments require continuous administration. The physician monitors CD4 counts and uses flow cytometry every other month, followed by gene rearrangement testing to confirm minimal residual disease (MRD) when flow cytometry becomes negative.

    When patients experience breakthrough on mogamulizumab or initial treatment failure, the treatment algorithm includes HDAC inhibitors for continued blood involvement, photopheresis for lower disease volume, and occasionally interferons. Alemtuzumab serves as another option, though timing considerations are crucial since many younger patients with Sézary syndrome eventually require donor stem cell transplantation. Mogamulizumab use prior to transplant increases graft-ca-host disease risk, making treatment sequencing critical. Romidepsin emerges as the preferred choice for patients with nodal disease or significant skin involvement, offering effective blood clearance with published data supporting reduced frequency maintenance dosing.

    Advanced treatment strategies involve combination approaches with mogamulizumab as the backbone therapy. Physicians successfully combine mogamulizumab with extracorporeal photopheresis (ECP) for blood breakthrough, bexarotene for additional skin response, and low-dose interferon for synergistic effects. These combination therapies demonstrate additive effects across different disease compartments. The multidisciplinary approach proves essential for managing complex cases, particularly when differentiating between treatment response and disease progression in various anatomical sites including blood, skin, and lymph nodes.

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