Oral Presentation 18th International Congress on Photobiology 2024

Phototherapy of cutaneous T-cell lymphomas (#156)

Franz Trautinger 1 2
  1. Karl Landsteiner Society, Institute of Dermatological Research, St. Poelten, Austria
  2. Dept. of Dermatology and Venereology, Karl Landsteiner University of Health Sciences, St. Poelten, Austria

Mycosis fungoides (MF), the most common variant among cutaneous T-cell lymphomas (CTCL), is characterized in its early stages by clonal proliferation of malignant T-cells in the skin, which manifest with erythematous patches and plaques. In some patients, progression occurs during the chronic course with cutaneous tumors and involvement of extracutaneous organs. Skin-directed therapies (SDT) are primarily used in the early stages of the disease. Among SDT, phototherapy with ultraviolet A radiation in combination with 8-methoxypsoralen (PUVA) and ultraviolet B radiation (UVB) have a long tradition in the treatment of MF and are highly effective in achieving remission. Sézary syndrome (SS) is a rare and more aggressive CTCL variant with generalized skin involvement. Patients with SS and with erythroderma from MF can benefit from treatment with extracorporeal photochemotherapy (ECP) where peripheral blood is exposed to PUVA. Another photoresponsive CTCL variant is lymphomatoid papulosis (LP), a CD30+ lymphoproliferative disease characterised by chronically recurring papules. LP responds favourably to PUVA but low dose methotrexate might be preferred for long term disease control. Phototherapy can be safely combined with systemic agents, especially interferon-alpha and retinoids.Recently, updated treatment guidelines have been published that include evidence-based algorithms for the stage-oriented treatment of MF. PUVA and narrow-band UVB (NB-UVB) are recommended as initial treatment for early stages, while combination treatments are reserved for refractory and more advanced cases; ECP is mentioned among the standard treatments for MF erythroderma and SS. Uncertainties exist regarding optimized treatment dose and schedules, the use of phototherapy for maintenance, and the role of newer phototherapeutic modalities (e.g. ultraviolet A1 radiation, excimer sources, photodynamic therapy) in the treatment of CTCL.