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International Prognostic Scoring System for Mastocytosis (IPSM): A retrospective cohort study

The Lancet Haematology Nov 12, 2019

Sperr WR, Kundi M, Alvarez-Twose I, et al. - With an intent to help improve prognostication for patients with mastocytosis, researchers developed as well as sought validation for a prognostic score for these patients. For this purpose, they studied data of the registry of the European Competence Network on Mastocytosis incorporating 1,639 patients (age 17–90 years) diagnosed with mastocytosis according to WHO criteria between Jan 12, 1978, and March 16, 2017, and determined prognostic variables predicting survival outcomes. They verified the 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. The prognostic value of the WHO classification was validated. Classification of patients with non-advanced mastocytosis into low (no risk factors), intermediate 1 (one risk factor), and intermediate 2 (two risk factors) groups was enabled by scoring system constructed based on independent prognostic variables for overall survival identified in patients with non-advanced mastocytosis. A separate score for advanced mastocytosis with four risk categories was also built. The validation cohort with 413 patients with non-advanced disease and 49 with advanced mastocytosis confirmed the prognostic value of both scores. Overall, the utility of the IPSM scores, for patients with non-advanced and advanced mastocytosis, in predicting survival outcomes and guiding treatment decisions was suggested. However, future trials to confirm the predictive value of the IPSM are required.
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