Carter et al reported the NIH Idiopathic Clonal Anaphylaxis Score (NICAS) to evaluate patients with idiopathic anaphylaxis. This can help to identify a patient who should have a bone marrow biopsy to identify a monoclonal mast cell disorder. The authors are from the National Institute of Allergy and Infectious Diseases at NIH and the Food and Drug Administration.
Patient selection: idiopathic anaphylaxis with >= 3 episodes per year
Prevalence of clonal mast cell disorder: 14%
Parameters:
(1) gender
(2) clinical symptoms
(3) serum tryptase concentration in ng/mL
(4) allele-specific PCR
Parameter
Finding
Points
gender
male
1
female
-1
clinical symptoms
angioedema absent
1
angioedema present
0
flushing
-1
no flushing
0
urticaria
1
no urticaria
0
syncope
3
no syncope
0
serum tryptase
< 11.4 ng/mL
-1
>= 11.4 ng/mL
1
allele specific PCR
negative
-1
`
positive
3
where:
• The scoring of clinical symptoms is ambiguous. One option is that only one item is selected. Since these are non-exclusive options it was elected to implement each separately. The range of values is -1 to 3 for the first option and -1 to 5 for the second.
total score =
= SUM(points for all 4 parameters)
Interpretation:
• minimum score: -4
• maximum score: 10
• A score >= 2 indicates a clonal mast cell disorder and a bone marrow is indicated.
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