Mast Cell Activation Syndromes: Comparison Between Two Scoring Models to Predict for Mast Cell Clonality

Data de publicação: Data Ahead of Print:

Autores da FMUP

  • Tiago Emanuel Azenha Rama

    Autor

  • André Miguel Afonso De Sousa Moreira

    Autor

Participantes de fora da FMUP

  • Torrado, I
  • Henriques, AF
  • Sanchez-Mu?oz, L
  • Jara-Acevedo, M
  • Navarro-Navarro, P
  • Caldas, C
  • Mayado, A
  • Mu?oz-Gonz?lez, J
  • García-Montero, A
  • Mollejo, M
  • Redondo, E
  • Garbán, A
  • Orlao, A
  • Alvarez-Twose, I

Unidades de investigação

Abstract

BACKGROUND: The Red Espanola de Mastocitosis (Spanish Network on Mastocytosis) score (REMAs) and the National Institutes of Health idiopathic clonal anaphylaxis score (NICAS) were developed for more efficient screening of mast cell (MC) clonality in MC activation syndromes. In a limited idiopathic anaphylaxis case series, the NICAS showed higher accuracy compared with the REMAs. OBJECTIVE: To compare the performance of the REMAs against the NICAS in the diagnosis of MC clonality.METHODS: We compared the diagnostic value of the REMAs against the NICAS in 182 patients (63% men, median age 56 years) who presented with anaphylaxis triggered by Hymenoptera venom allergy (45%), drugs (15%), food (11%), idiopathic anaphylaxis (20%), and mixed causes (10%). KIT mutation was assessed in parallel in whole blood and bone marrow (BM) and, when negative, in highly purified BM MC. TPSAB1 was genotyped in a subset of 71 patients. RESULTS: We found higher accuracy and rates of correctly classified patients for the REMAs (82% and 84%) compared with the NICAS (75% and 75%; P = .02 and P = .03, respectively), particularly among men (P = .05), patients with systemic mastocytosis (P = .05), those presenting anaphylaxis owing to any cause featuring urticaria (P = .04), cardiovascular symptoms (P = .02), and/or presyncope (P = .02) and those with a blood-negative/BM-positive KIT mutational profile (P = .002), but not hereditary a-tryptasemiaeassociated genotypes. Combined assessment of the REMAs and KITD816V in blood yielded an overall improved classification efficiency of 86% versus 84% for REMAs.CONCLUSIONS: The combined use of the REMAs and blood detection of KITD816V is recommended, but more sensitive blood-based molecular assays to detect KITD816V are needed. (c) 2022 American Academy of Allergy, Asthma & Immunology (J Allergy Clin Immunol Pract 2023

Dados da publicação

ISSN/ISSNe:
2213-2198, 2213-2201

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE  Elsevier

Tipo:
Article
Páginas:
908-9194
Link para outro recurso:
www.scopus.com

Citações Recebidas na Web of Science: 3

Citações Recebidas na Scopus: 9

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Keywords

  • Anaphylaxis; Mast cell activation syndromes; Mas-tocytosis; KIT mutation; Hereditary a-tryptasemia

Financiamento

Proyectos asociados

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