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Mast Cell Activation Syndromes: Comparison Between Two Scoring Models to Predict for Mast Cell Clonality
Tiago Azenha Rama1, Inés Torrado2, Ana Filipa Henriques3
1Serviço de Imunoalergologia, Centro Hospitalar Universitário São João, Porto, Portugal; Serviço de Imunologia Básica e Clínica, Departamento de Patologia, Faculdade de Medicina da Universidade do Porto, Porto, Portugal; EPIUnit, Institute of Public Health, University of Porto, Porto, Portugal; Laboratory for Integrative and Translational Research in Population Health, Porto, Portugal.
The Red Española de Mastocitosis (REMAs) score is more accurate than the National Institutes of Health idiopathic clonal anaphylaxis score (NICAS) for diagnosing mast cell clonality. Combining REMAs with KIT mutation testing improves classification efficiency.
Area of Science:
- Immunology
- Genetics
- Oncology
Background:
- Mast cell (MC) activation syndromes require efficient screening for MC clonality.
- The Red Española de Mastocitosis (REMAs) score and National Institutes of Health idiopathic clonal anaphylaxis score (NICAS) were developed for MC clonality screening.
- Previous studies suggested NICAS may be more accurate than REMAs in idiopathic anaphylaxis.
Purpose of the Study:
- To compare the diagnostic performance of REMAs and NICAS in identifying MC clonality.
- To evaluate the accuracy of REMAs and NICAS across various anaphylaxis triggers and patient demographics.
Main Methods:
- A comparative study involving 182 patients presenting with anaphylaxis.
- Assessment of REMAs and NICAS diagnostic values.
- KIT mutation analysis in blood and bone marrow (BM), with TPSAB1 genotyping in a subset.
- Analysis of diagnostic accuracy based on anaphylaxis cause, symptoms, and mutational profiles.
Main Results:
- REMAs demonstrated higher accuracy (82%) and patient classification rates (84%) compared to NICAS (75% and 75%; P=.02 and P=.03).
- REMAs showed superior performance in men, patients with systemic mastocytosis, and those with urticaria, cardiovascular symptoms, or presyncope.
- Combined REMAs and blood KITD816V testing achieved 86% classification efficiency, outperforming REMAs alone (84%).
Conclusions:
- REMAs is a more accurate tool than NICAS for diagnosing MC clonality in patients with anaphylaxis.
- Combined assessment of REMAs and blood KITD816V mutation testing is recommended for improved diagnostic efficiency.
- Development of more sensitive blood-based molecular assays for KITD816V detection is needed.
