Description

Alvarez-Twose et al reported a score for predicting a clonal mast cell disorder in a patient with symptoms of systemic mast cell activation. The authors are from Hospital Virgen del Valle in Toledo, Spain.


The Spanish Network of Mastocytosis is known as REMA.

 

Parameters:

(1) sex

(2) urticaria, pruritis and/or angioedema

(3) presyncope and/or syncope

(4) serum tryptase at baseline

 

Parameter

Finding

Points

sex

male

1

 

female

-1

urticaria, pruritis and/or angioedema

none

1

 

any

-2

presyncope and/or syncope

no

0

 

yes

3

serum tryptase

< 15 ng/mL

-1

 

15 to 25 ng/mL

0

 

> 25 ng/mL

2

 

total score =

= SUM(points for all of the parameters)

 

Interpretation:

• minimum score: -4

• maximum score: 7

• A score < 2 is associated with a low probability of clonal MCAD.

• A score >= 2 is associated with a high probability of clonal MCAD

 

Performance:

• The sensitivity was 0.92 and specificity 0.81.


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