cliot (version 1.0.0)

rema_mastocytosis_score: Spanish Network on Mastocytosis (REMA) Score

Description

Calculates the REMA Score to predict the presence of clonal mast cell disease (Systemic Mastocytosis or Monoclonal Mast Cell Activation Syndrome) in patients with mast cell activation symptoms but without skin lesions (systemic mastocytosis sine urticaria pigmentosa). A score of 2 or higher suggests a higher probability of clonality and may warrant bone marrow biopsy.

Usage

rema_mastocytosis_score(sex, clinical_symptoms, tryptase_level)

Value

A list containing:

REMA_Score

The calculated total score (Range -8 to +7).

Prediction

Interpretation of probability (Low < 2, High >= 2).

Test_Characteristics_at_Cutoff_2

Sensitivity and Specificity metrics.

Arguments

sex

String. Patient sex ("male" or "female"). Male: +2 points. Female: -4 points.

clinical_symptoms

String. The primary clinical presentation. "pruritus_urticaria_angioedema": Pruritus, urticaria, or angioedema (-4 points). "syncope_presyncope": Syncope or presyncope (+3 points). "anaphylaxis": Anaphylaxis (+1 point). "other": Other symptoms (0 points).

tryptase_level

Numeric. Baseline serum tryptase level in ng/mL. < 15: 0 points. 15 - 24.9: +1 point. >= 25: +2 points.

References

Alvarez-Twose I, González-de-Olano D, Sánchez-Muñoz L, et al. Clinical, biological, and molecular characteristics of clonal mast cell disorders presenting with systemic mast cell activation symptoms. J Allergy Clin Immunol. 2010;125(6):1269-1278.e2. doi:10.1016/j.jaci.2010.02.019

Examples

# Example 1: High Probability
# Male (+2), Syncope (+3), Tryptase 20 (+1)
# Score = 6
rema_mastocytosis_score("male", "syncope_presyncope", 20)

# Example 2: Low Probability
# Female (-4), Urticaria (-4), Tryptase 10 (0)
# Score = -8
rema_mastocytosis_score("female", "pruritus_urticaria_angioedema", 10)