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The REMA Score Revisited: An Optimized Algorithm for Early Prediction of Clonal Mast Cell Disease
Irina Sucre-Adrianza1, Horacio Caligaris1,2,3,4, Cristina Morales-Cabeza1,2,3,4
1Institute of Mastocytosis Studies of Castilla-La Mancha (CLMast)-Spanish Reference Center of Mastocytosis, Hospital Virgen del Valle, Toledo, Spain.
Allergy
|May 19, 2026
Summary
The REMA score effectively detects clonal mast cell activation disorders without skin involvement but needs refinement. An updated algorithm improves specificity for selecting patients for bone marrow examination.
Area of Science:
- Allergy and Immunology
- Hematology
- Oncology
Background:
- The Spanish Network on Mastocytosis (REMA) score aids in diagnosing clonal mast cell activation disorders (MCAD) in patients with anaphylaxis but without skin mastocytosis (MIS).
- This study aimed to re-evaluate the REMA score's diagnostic accuracy after 15 years and propose improvements.
Purpose of the Study:
- To reassess the diagnostic accuracy of the REMA score for clonal MCAD in patients without MIS.
- To refine the REMA score by incorporating additional predictors to optimize patient selection for bone marrow (BM) examination.
Main Methods:
- Retrospective analysis of 1204 patients with severe MC mediator-related symptoms without MIS.
- Evaluation included clinical assessment, serum tryptase, BM histology, immunophenotyping, and KIT mutational analysis.
- Multivariable analysis identified independent predictors to refine the diagnostic algorithm.
Main Results:
- Clonal MCAD was diagnosed in 64% of patients undergoing BM study.
- The REMA score demonstrated high sensitivity (87%-90%) but low specificity (34%-35%).
- An updated algorithm incorporating elevated tryptase and insect venom triggers, along with KIT p.D816V detection, improved specificity to 57% while maintaining 86% sensitivity.
Conclusions:
- The REMA score is highly sensitive for detecting clonal MCAD without MIS.
- An enhanced diagnostic algorithm incorporating clinical and molecular markers improves specificity for selecting patients with low REMA scores (0-2) for BM evaluation.
