PROBABILITY OF CLONAL MAST CELS IN PATIENTS PRESENTING WITH ANAPHYLAXIS IN THE ABSENCE OF SKIN MASTOCYTOSIS (NICAS score)
Patients with clonal mast cell disease can present as having idiopathic anaphylaxis (IA). The NIH Idiopathic Clonal Anaphylaxis Score (NICAS) can be used to select those patients more likely to have an underlying clonal mast cell disorder (monoclonal mast cell activation syndrome or systemic mastocytosis) and thus candidates for a bone marrow biopsy.
 
Research authors: Carter MC, Desai A, Komarow HD, Bai Y, Clayton ST, Clark AS, Ruiz-Esteves KN, Long LM, Cantave D, Wilson TM, Scott LM, Simakova O, Jung MY, Hahn J, Maric I, Metcalfe DD.
General details Custom formula Study characteristics Files & References Validations
Model author
Model ID
1014
Version
1.25
Revision date
2017-12-01
Medical specialty
MeSH terms
  • Anaphylaxis
  • Mastocytosis
  • Clinical Prediction Rule
  • Model Type
    Linear model (Calculation)
    Status
    public
    Rating
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    Formula
    No Formula defined yet
    Condition Formula

    Additional information

    Abstract original reserach: 

    Background:
    Clonal mast cell disorders occur in a subset of patients with systemic reactions to Hymenoptera stings. This observation has prompted the question of whether clonal mast cell disorders also occur in patients with idiopathic anaphylaxis (IA).

    Objective:
    We sought to determine the prevalence of clonal mast cell disorders among patients with IA, criteria to identify those patients who require a bone marrow biopsy, and whether the pathogenesis of IA involves a hyperresponsive mast cell compartment.

    Methods:
    We prospectively enrolled patients with IA (>3 episodes per year) who then underwent a medical evaluation that included a serum tryptase determination, allele-specific quantitative PCR (ASqPCR) for the KIT D816V mutation, and a bone marrow examination. Mast cells were cultured from peripheral blood CD341 cells and examined for releasability after FcεRI aggregation.

    Results:
    Clonal mast cell disease was diagnosed in 14% of patients referred with IA. ASqPCR for the KIT D816V mutation was a useful adjunct in helping identify those with systemic mastocytosis but not monoclonal mast cell activation syndrome. A modified overall clonal prediction model was developed by using clinical findings, a serum tryptase determination, and ASqPCR. There was no evidence of a hyperresponsive mast cell phenotype in patients with IA.

    Conclusion:
    Patients with clonal mast cell disease can present as having IA. Distinct clinical and laboratory features can be used to select those patients more likely to have an underlying clonal mast cell disorder (monoclonal mast cell activation syndrome or systemic mastocytosis) and thus candidates for a bone marrow biopsy.

    Source: 
    1. Carter MC, Desai A, Komarow HD, Bai Y, Clayton ST, Clark AS, et al. A distinct biomolecular profile identifies monoclonal mast cell disorders in patients with idiopathic anaphylaxis. J Allergy Clin Immunol. 2017. pii: S0091-6749(17)30995-8. doi: 10.1016/j.jaci.2017.05.036.

    Study Population

    Total population size: 37

    Additional characteristics

    No additional characteristics defined
    No public validations available

    Total NIH idiopathic clonal anaphylaxis score (NICAS) :
    ... points

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    Result

    Total NIH idiopathic clonal anaphylaxis score (NICAS) : points

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    Outcome stratification

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    Conditional information

    Result interpretation

    How this model should be used;
    Patients with clonal mast cell disease can present as having idiopathic anaphylaxis (IA). Distinct clinical and laboratory features can be used to select those patients more likely to have an underlying clonal mast cell disorder (monoclonal mast cell activation syndrome or systemic mastocytosis) and thus candidates for a bone marrow biopsy.

    Context information
    The NICAS algorithm was specifically developed for the evaluation of patients presenting with severe recurrent idiopathic anaphylaxis (IA) and thus is not meant to replace previous scoring systems. In the underling patient cohort (n=37), no patients with IA would have undergone an unnecessary bone marrow procedure, and all patients with ISM would have received a correct diagnosis, as would have 75% of patients with monoclonal mast cell activation syndrome (MMAS).

    Source:

    1. Carter MC, Desai A, Komarow HD, Bai Y, Clayton ST, Clark AS, et al. A distinct biomolecular profile identifies monoclonal mast cell disorders in patients with idiopathic anaphylaxis. J Allergy Clin Immunol. 2017. pii: S0091-6749(17)30995-8. doi: 10.1016/j.jaci.2017.05.036.

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    Calculations alone should never dictate patient care, and are no substitute for professional judgement. See our full disclaimer.

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