International prognostic scoring system for mastocytosis (IPSM): a retrospective cohort study

Publication date: Available online 31 October 2019Source: The Lancet HaematologyAuthor(s): Wolfgang R Sperr, Michael Kundi, Ivan Alvarez-Twose, Bjorn van Anrooij, Joanna N G Oude Elberink, Aleksandra Gorska, Marek Niedoszytko, Karoline V Gleixner, Emir Hadzijusufovic, Roberta Zanotti, Patrizia Bonadonna, Massimiliano Bonifacio, Cecelia Perkins, Anja Illerhaus, Chiara Elena, Serena Merante, Khalid Shoumariyeh, Nikolas von Bubnoff, Roberta Parente, Mohamad JawharSummaryBackgroundThe WHO classification separates mastocytosis into distinct variants, but prognostication remains a clinical challenge. The aim of this study was to improve prognostication for patients with mastocytosis.MethodsWe analysed data of the registry of the European Competence Network on Mastocytosis including 1639 patients (age 17–90 years) diagnosed with mastocytosis according to WHO criteria between Jan 12, 1978, and March 16, 2017. Univariate and multivariate analyses with Cox regression were applied to identify prognostic variables predicting survival outcomes and to establish a prognostic score. We validated this International Prognostic Scoring System in Mastocytosis (IPSM) with data of 462 patients (age 17–79 years) from the Spanish network Red Española de Mastocitosis diagnosed between Jan 22, 1998, and Nov 2, 2017.FindingsThe prognostic value of the WHO classification was confirmed in our study (p<0·0001). For patients with non-advanced mastocytosis (n=1380), we identified age 60 years or o...
Source: The Lancet Haematology - Category: Hematology Source Type: research