How we treat advanced stage cutaneous T‐cell lymphoma – mycosis fungoides and Sézary syndrome

Abstract

T-cell lymphomas (TCLs) constitute a rare subset of non-Hodgkin lymphomas, with mycosis fungoides/S ezary syndrome (MF/SS) being the most common subtype of cutaneous TCLs (CTCLs). Considered an incurable but treatable disease, MF/SS management presents several challenges including diagnostic delays, debilitating effect on patients' quality of life, need for several lines of therapies, multidisciplinary care and cumulative drug toxicities limiting duration of use. The present review intends to provide an overview of the recent advances in our understanding of the biology of CTCL and how these are being leveraged to provide additional treatment options for management of advanced and recurrent disease. In addition, the discussion of the different modalities of treatment is summarised to further outline the importance of multidisciplinary care and early referral to CTCL centres.

Mode of action, indications and recommendations on extracorporeal photopheresis (ECP)

“…Depending on the stage, different therapeutic strategies are pursued, starting with topical therapies, phototherapy (narrowband UVB or PUVA), up to systemic treatments for advanced stages with interferon or bexarotene 26 . Recent therapeutic approaches involve the use of Brentuximab, Mogalizumab or PD1 antibodies 27,28 …”

“…26 Recent therapeutic approaches involve the use of Brentuximab, Mogalizumab or PD1 antibodies. 27,28 The first clinical publication on the innovative therapy option with ECP was, as mentioned above, on advanced, therapy-resistant CTCL patients. Of 37 patients, a treatment response of 73% was achieved, which was demonstrated by an improvement of skin symptoms by an average of 64%.…”

Proposal of a comprehensive model for palliative therapeutic interventions in patients with mycosis fungoides and Sézary syndrome

“…The optimal management is not clear here, but for young patients, systemic chemotherapy should be used, and autologous or allogeneic transplantation should be considered, especially for high-risk disease. Consolidative radiation can be an option for local transformations 6,21,23 .…”

“…Patients with extensive large cell transformation involvement or aggressive clinical skin involvement may receive combination therapy. Autologous hematopoietic stem cell transplantation can be an option for patients with complete responses, and allogeneic transplantation in patients without complete responses (young patients, aggressive clinical disease, or after exhausting other options) 23,24 .…”

Decoding Early Mycosis Fungoides: Histopathologic and Immunohistochemical Clues

“…Histopathologically, the lesions of MF present a range of features, progressing from confined epidermotropic, folliculotropic, and/or syringotropic lymphoid infiltrates in the initial phases to densely distributed dermal and subcutaneous infiltrates lacking prominent epidermotropism in the later stages of the disease [ 4 , 5 ]. Recognizing the early manifestations of eMF, such as patch or early plaque, poses a substantial diagnostic challenge.…”