Guidelines for mycosis fungoides and Sézary syndrome clinical trials were published in 2011 to standardize endpoint criteria and trial design. Our retrospective cohort study of mycosis fungoides/Sézary syndrome clinical trials registered on ClinicalTrials.gov and pivotal trials supporting drug approvals and label extensions evaluates adherence to these guidelines. Sixty-three trials met our inclusion criteria. In a subpopulation of trials, mean adherence to the guidelines was approximately 60%. When comparing trials that began in the first 6 years after their publication with those that started after, we found no difference in mean adherence (4.12 vs 3.41) (P = .15). Among the 8 pivotal trials supporting new mycosis fungoides or Sézary syndrome systemic therapies from 1990 to 2020, systemic trials published after 2011 were more likely to randomize patients (100 vs 0%, P = .036), perform superiority testing (100 vs 0%, P = .036), and use an intention-to-treat analysis (100 vs 0%, P = .036). The design of trials registered on Clinicaltrials.gov did not change significantly between the first 6 years after the publication of the guidelines and after. This demonstrates that the guidelines are still not consistently implemented across all trials. However, registrational trials were more likely to implement the recommendations.

Extracorporeal photopheresis (ECP) has been used for over 35 years in the treatment of erythrodermic cutaneous T-cell lymphoma (CTCL) and over 20 years for chronic and acute graft-versus-host disease (GvHD) and solid organ transplant rejection. ECP for CTCL and GvHD is available at specialised centres across the UK. The lack of prospective randomised trials in ECP led to the development of UK Consensus Statements for patient selection, treatment schedules, monitoring protocols and patient assessment criteria for ECP. The recent literature has been reviewed and considered when writing this update. Most notably, the national transition from the UVAR XTS® machine to the new CELLEX machine for ECP with dual access and a shorter treatment time has led to relevant changes in these schedules. This consensus statement updates the previous statement from 2007 on the treatment of CTCL and GvHD with ECP using evidence based medicine and best medical practise and includes guidelines for both children and adults.

BACKGROUND: Ultraviolet light (UVL) is a long established treatment for mycosis fungoides (MF) and Sézary syndrome (SS), subtypes of cutaneous T-cell lymphoma (CTCL). Treatments have traditionally included broadband, narrowband ultraviolet B light (UVB) and psoralen plus ultraviolet A light photochemotherapy (PUVA), but more recently, treatment options have expanded to include UVA1 and excimer laser. UVL is used either as monotherapy or as an adjuvant to systemic therapy, demonstrating efficacy in many cases that equal or surpass systemic medications. Despite its utility and duration of use, the current practice of using UVL guidelines for psoriasis to treat patients with MF/SS is problematic because the goals of prolonging survival and preventing disease progression are unique to CTCL compared to psoriasis.
OBJECTIVE: We sought to develop separate guidelines for phototherapy for MF/SS for both clinical practice and for clinical trials.
METHODS: Literature review and cutaneous lymphoma expert consensus group recommendations.
RESULTS: This paper reviews the published literature for UVB and UVA/PUVA in MF/SS and suggests practical standardized guidelines for their use.
CONCLUSIONS: New standardization of phototherapy.
CONCLUSIONS: These guidelines should allow the comparison of results with phototherapy in MF/SS across different stages of patients, centers, and in combination with other agents in practice and in clinical trials.

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