title: Evaluation of the WHO criteria for the classification of patients with mastocytosis.
abstract: Diagnosis and classification of mastocytosis is currently based on the World Health Organization (WHO) criteria. Here, we evaluate the utility of the WHO criteria for the diagnosis and classification of a large series of mastocytosis patients (n=133), and propose a new algorithm that could be routinely applied for refined diagnosis and classification of the disease. Our results confirm the utility of the WHO criteria and provide evidence for the need of additional information for (1) a more precise diagnosis of mastocytosis, (2) specific identification of new forms of the disease, (3) the differential diagnosis between cutaneous mastocytosis vs systemic mastocytosis, and (4) improved distinction between indolent systemic mastocytosis and aggressive systemic mastocytosis. Based on our results, a new algorithm is proposed for a better diagnostic definition and prognostic classification of mastocytosis, as confirmed prospectively in an independent validation series of 117 mastocytosis patients.
authors.affiliation: Instituto de Estudios de Mastocitosis de Castilla La Mancha, Hospital Virgen del Valle, Toledo, Spain. sanchezlaura99@gmail.com
authors.forename: Laura
authors.initials: L
authors.lastname: Sánchez-Muñoz
authors.forename: Ivan
authors.initials: I
authors.lastname: Alvarez-Twose
authors.forename: Andrés C
authors.initials: AC
authors.lastname: García-Montero
authors.forename: Cristina
authors.initials: C
authors.lastname: Teodosio
authors.forename: María
authors.initials: M
authors.lastname: Jara-Acevedo
authors.forename: Carlos E
authors.initials: CE
authors.lastname: Pedreira
authors.forename: Almudena
authors.initials: A
authors.lastname: Matito
authors.forename: Jose Mario T
authors.initials: JM
authors.lastname: Morgado
authors.forename: Maria Luz
authors.initials: ML
authors.lastname: Sánchez
authors.forename: Manuela
authors.initials: M
authors.lastname: Mollejo
authors.forename: David
authors.initials: D
authors.lastname: Gonzalez-de-Olano
authors.forename: Alberto
authors.initials: A
authors.lastname: Orfao
authors.forename: Luis
authors.initials: L
authors.lastname: Escribano
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000465:Algorithms; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D008415:Mastocytosis / Q000145:classification* / Q000175:diagnosis*; D008875:Middle Aged; D014944:World Health Organization; D055815:Young Adult