A New Prognostic Index (CLIPI) for Advanced Cutaneous Lymphoma Enables Precise Patient Risk Stratification.
Scarisbrick JJ., Quaglino P., Whittaker SJ., Bagot M., Guenova E., Papadavid E., Prince HM., Sanches JA., Miyashiro D., Servitje O., Querfeld C., Akilov OE., Arumainathan A., Bennett L., Battistella M., Benstead K., Berti EF., Beylot-Barry M., Busschots A-M., Cowan RA., Dummer R., Dunnill G., Estrach MT., Evison F., Bashir AE., Geskin LJ., Gru A., Guitart J., Herrera MC., Hodak E., Horwitz SM., Jonak C., Klemke C-D., Knobler R., McKay P., Marschalkó M., Massone C., Matin R., Mitteldorf C., Novoa RA., Ortiz-Romero PL., Pimpinelli N., Pujol RM., Ranki A., Vankeva L., Rieger KE., Stadler R., Trautinger F., Latzka J., Vermeer MH., Wachsmuth R., Weatherhead S., Wehkamp U., Wobser M., Kim YH.
Advanced mycosis fungoides (MF) and Sézary syndrome (SS) have a poor prognosis with overall survival <5 years. Studies have found the current clinical staging (IA-IVB) inadequate for risk stratification. Developing a prognostic index in MF/SS will identify patients with poor outcomes and may allow better management decisions and improved survival.PROCLIPI (Prospective Cutaneous Lymphoma International Prognostic Index) Study was launched in 2015 at 46 international expert MF/SS Centers, prospectively collecting pre-defined datasets in newly diagnosed MF/SS patients to determine a cutaneous lymphoma IPI (CLIPI).552 advanced stage MF/SS patients were recruited. The 5-year overall survival (OS) was IIB=50.0%, IIIA=64.8%, IIIB=43.9%, IVA1=50.8%, IVA2=25.9%, IVB=36.9%. Factors at diagnosis associated with a significantly worse survival were N3 status; p<0.001, age>60yrs; p<0.001, raised serum lactate dehydrogenase; p=0.005 and large-cell transformation in skin; p=0.006. Modelling these 4 independent risk-factors into a CLIPI found there was a statistically significant worse OS in high-versus low-risk p<0.001, high-versus intermediate-risk p=0.002 as well as intermediate-versus low-risk p=0.010. 5 Year OS were 63.3%, 44.7% and 18.3% in the low-, intermediate- and high-risk PROCLIPI cohort respectively.In advanced stage MF/SS there was a low 5-year survival rate and increasing stage was not associated with worsening survival. The use of CLIPI to stratify patients into low, intermediate, and high-risk prognostic groups has the potential to improve patient outcomes by helping guide optimal treatment selection. CPMS ID 17662 (PROCLIPI), RRK4970, ClinicalTrials.Gov ID: NCT02848274.