DISCLAIMER:
This survival risk assessment tool is designed to assist healthcare professionals in estimating the prognosis of patients with advanced systemic mastocytosis associated with KIT D816V mutation, using the Revised Mutation-Adjusted Risk Score (MARS-R). The MARS-R can be applied at the initiation of treatment with the KIT inhibitors midostaurin or avapritinib. MARS-R risk estimates and survival projections are derived from aggregated data and model-based assumptions, and it does not substitute clinical judgment.
For ASXL1, RUNX1, and SETBP1, only Tier 1 and Tier 2 mutations are considered. To minimize the influence of outliers on model performance, age was truncated to a range of 38 to 86 years (1st to 99th percentile), monocyte counts were truncated at 9 × 109/L (99th percentile), and platelet counts were truncated at 250 × 109/L (80th percentile). Values outside these ranges did not significantly affect model predictions and were therefore adjusted accordingly.
This model is based on the evidence and standard of care at the time of its development. Future clinical data, emerging prognostic markers, and new therapeutic options may change its applicability or accuracy. The developers assume no responsibility for clinical decisions.
Developed by the Medical Faculty Mannheim of Heidelberg University in cooperation with Blueprint Medicines Corporation.
Contact us: If you have any questions or suggestions about the MARS-R, please contact:
- Johannes Lübke, MD (johannes.luebke@medma.uni-heidelberg.de)
- Andreas Reiter, MD (andreas.reiter@medma.uni-heidelberg.de)