Mycosis fungoides (MF) is the most prevalent form of primary cutaneous T-cell lymphoma. Patients frequently present with erythematous scaly patches in early-stage disease typically associated with a favourable prognosis and survival of 10–35 years, but over 25% may progress to advanced disease with nodal or systemic involvement and a median survival of less than 4 years and 30% present with the advanced stages. Identifying prognostic factors in MF may allow better management and improve survival and the development of a prognostic index should help identify patients at risk of progression.
In order to investigate prognostic factors the PROCLIPI Study was launched in July 2015; The PROCLIPI study is the PROspective Cutaneous Lymphoma International Study for the development of a prognostic index in mycosis fungoides. This study is funded in Europe with an EADV Project based grant and a Cancer Research UK Population Grant. The study prospectively collects clinical, pathological, genotypic, treatment and quality of life data on MF/SS from worldwide sites. The study tests prognostic parameters separately in early stage MF and advanced stage MF/Sezary syndrome (led by Youn Kim) in order to identify prognostic factors which are important predictors of outcome in MF/SS with the aim of improving survival and quality of life. The goal is to recruit 1000 patients with early stages of mycosis fungoides and 500 with advanced stages over 5 years. Thus far 693 patients have been recruited from 42 international Centres, Fig 1. (540 early and 153 late stage patients) including a male predominance as previously suspected; 444 male and 249 female patients (ratio 1.8:1).
A central review process of clinicopathological data is led by Rein Willemze to confirm correct diagnosis and stage. Treatment data is being collected prospectively to determine response rates and duration and to identify the most efficacious treatments for stage, led by Pietro Quaglino. A Federated Biobank, led by Maarten Vermeer, detailing tissue samples for future translational projects has over 500 registered samples.
Already we are looking at trends in possible prognostic factors and time to progression. By the end of 5 years we will be able to model and test a prognostic index in advanced disease. Whilst recruitment of early stage patients will stop at 5-year survival data will be continued to be accrued for a further 10 years.
Dr. Julia Scarisbrick