PROCLIPI facilitating international research in rare cancers: a web-based data system designed to collect clinicalpathological, radiological, treatment and quality of life data in cutaneous lymphoma

Julia Scarisbrick (Chair EORTC Cutaneous Lymphoma Taskforce), Pietro Quaglino (Past Chair)

PROCLIPI is a web-based secure database system which is specifically designed to collect information on clinical, immunohistochemical and radiological data for cutaneous lymphoma. Meaningful information on rare diseases can only be achieved using prospective international data and this easily accessible system allows multiple centres to enter data with the anonymised data pooled centrally for analysis and research.

The PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study was launched in 2015 with the long-term goal of developing a prognostic index for cutaneous lymphoma. In addition to the clinicopathological and genotypic data, treatment responses and quality of life are being collected, alongside a federated tissue biobank for translational studies. Central clinicopathological review is performed to confirm diagnosis and stage.

Already 836 patients have been registered on PROCLIPI from 46 sites over 5 continents, and this includes 600 early stage (IA-IIA) patients. The data confirm the predominance of males (1.7:1) and have shown that patients with early stage disease present at a younger age. Median age at diagnosis of early stage disease is 57 years, compared to 64yrs in those presenting in advanced stages (p<.0001). However, the median time of MF-like lesions prior to diagnosis is 36 months in both, confirming diagnostic delay and suggesting patients presenting with advanced disease are not undiagnosed early stage patients. Raised serum LDH & large cell transformation (LCT) in skin are among the candidate prognostic factors and have been shown to be associated with advanced disease. LCT has been recorded in 3% early stage & 25% advanced patients (p<0.001) whilst 15.7% early stage & 47% advanced stage patients have raised LDH at diagnosis (p<0.001). Tracking these patients for survival will give us important prognostic information.

Prospectively evaluating treatment responses with QOL to identify patients with a poorer prognosis will allow optimal treatments, better patient experience, and improve survival. Such information is only possible with global collaboration and PROCLIPI facilitates this data collection using well defined criteria. We believe that other rare diseases may benefit from this model.