A Cutaneous Lymphoma International Consortium 'Clic' Study Of Prognostic Parameters In Advanced Stages Of Mycosis Fungoides And Sezary Syndrome: Progress Towards Establishing A Prognostic Index To Augment Clinical Staging

Background: Cutaneous T cell lymphoma (CTCL) is a rare/orphan disease. Mycosis fungoides (MF) and Sézary syndrome (SS) are the major subtypes. Most patients with MF present in early stages (IA-IIA) with a good prognosis. However a third present in advanced stages (IIB-IVB) with a median survival of 1-5yrs. Clinical management is stage-based; however there is wide range of outcome within clinical stages. Currently, we lack evidence based prognostic information for further stratification that can optimize clinical management or be applied in therapeutic trials. Published prognostic studies in MF/SS have largely been single centre. A retrospective study from the UK & a US site proposed a potential prognostic model for MF/SS (Benton et al Eur J Cancer 2013;49:2859-68) but is limited in power and lacking standardised definitions. Due to the small numbers of patients presenting with advanced stage disease only a large collaboration would power a study to determine prognostic parameters with international relevance. Towards this aim, we have established an international collaborative alliance, the Cutaneous Lymphoma International Consortium (CLIC), to acts as an efficient conduit where well-defined parameters and end-points are used to design studies. This retrospective CLIC study tests selected prognostic parameters against survival in advanced stages of MF/SS in order to determine power and identify prognostic markers that impact globally and may be used in future prospective studies to establish an international prognostic index.