Criteria for the Regression of Pediatric Mastocytosis: A Long-Term Follow-Up.

Adult Child Follow-Up Studies Humans Longitudinal Studies Mast Cells Mastocytosis / diagnosis Mastocytosis, Systemic / diagnosis Mutation Proto-Oncogene Proteins c-kit / genetics
Cutaneous mastocytosis KIT mutation Mast cell Mast cell activation symptom Pediatric mastocytosis Prognosis Tryptase

Journal

The journal of allergy and clinical immunology. In practice
ISSN: 2213-2201
Titre abrégé: J Allergy Clin Immunol Pract
Pays: United States
ID NLM: 101597220

Informations de publication

Date de publication:
04 2021
Historique:
received: 25 03 2020
revised: 03 12 2020
accepted: 04 12 2020
pubmed: 19 12 2020
medline: 25 5 2021
entrez: 18 12 2020
Statut: ppublish

Résumé

Mastocytosis is a neoplastic condition characterized by the accumulation of mast cells (MCs) in 1 or more organ. Adults tend to have persistent, systemic mastocytosis, whereas MC infiltration in children is usually limited to the skin and typically regresses after several years. Both adults and children could display mast cell activation symptoms (MCASs) due to MC mediator release. In more than 85% of both adult and pediatric cases, KIT mutations are present, with the KIT D816V mutation being present in most affected adults but in only half the affected children. To identify the clinical, biological, and molecular factors associated with the regression of cutaneous mastocytosis (CM) in children, and to assess the correlation between MCASs and CM regression. Patients having suffered from pediatric-onset mastocytosis for at least 8 years were included in a longitudinal cohort study. Clinical data, the baseline serum tryptase level, the KIT sequence, and the progression of MCASs and CM were recorded. CM regressed in 210 of the 272 included patients (77.2%; mean time to regression, 6.10 years). The rare cases of aggressive systemic mastocytosis were symptomatic from the outset. Congenital mastocytosis and the KIT D816V mutation were associated with CM regression (odds ratio, 0.48, P = .031, and 0.173, P = .031, respectively). Aggravation of MCASs over time was correlated with the persistence of skin lesions. However, the MCASs became more intense in 19% of the patients with MCASs at baseline and CM regression, justifying long-term follow-up in this setting. Our results open up new hypotheses with regard to the spontaneous regression of CM in pediatric patients.

Sections du résumé

BACKGROUND
Mastocytosis is a neoplastic condition characterized by the accumulation of mast cells (MCs) in 1 or more organ. Adults tend to have persistent, systemic mastocytosis, whereas MC infiltration in children is usually limited to the skin and typically regresses after several years. Both adults and children could display mast cell activation symptoms (MCASs) due to MC mediator release. In more than 85% of both adult and pediatric cases, KIT mutations are present, with the KIT D816V mutation being present in most affected adults but in only half the affected children.
OBJECTIVE
To identify the clinical, biological, and molecular factors associated with the regression of cutaneous mastocytosis (CM) in children, and to assess the correlation between MCASs and CM regression.
METHODS
Patients having suffered from pediatric-onset mastocytosis for at least 8 years were included in a longitudinal cohort study. Clinical data, the baseline serum tryptase level, the KIT sequence, and the progression of MCASs and CM were recorded.
RESULTS
CM regressed in 210 of the 272 included patients (77.2%; mean time to regression, 6.10 years). The rare cases of aggressive systemic mastocytosis were symptomatic from the outset. Congenital mastocytosis and the KIT D816V mutation were associated with CM regression (odds ratio, 0.48, P = .031, and 0.173, P = .031, respectively). Aggravation of MCASs over time was correlated with the persistence of skin lesions. However, the MCASs became more intense in 19% of the patients with MCASs at baseline and CM regression, justifying long-term follow-up in this setting.
CONCLUSIONS
Our results open up new hypotheses with regard to the spontaneous regression of CM in pediatric patients.

Identifiants

DOI: 10.1016/j.jaip.2020.12.019 PMID: 33338682
pubmed: 33338682
pii: S2213-2198(20)31351-9
doi: 10.1016/j.jaip.2020.12.019
pii:
doi:

Substances chimiques

Proto-Oncogene Proteins c-kit EC 2.7.10.1

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1695-1704.e5

Informations de copyright

Copyright © 2020 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

Auteurs

Laura Polivka (L)

Department of Dermatology, Reference Center for Genodermatoses (MAGEC), Necker-Enfants Malades Hospital (AP-HP5), Paris-Centre University, Imagine Institute, INSERM U1163, Paris, France; Laboratory of Molecular Mechanisms of Hematologic Disorders and Therapeutic Implications, Necker Branch, INSERM U1163 and CNRS ERL 8654, Necker-Enfants Malades Hospital (AP-HP), Paris-Centre University, Imagine Institute, Paris, France; CEREMAST, Imagine Institute, INSERM U1163, Paris, France. Electronic address: laura.polivka@institutimagine.org.

Julien Rossignol (J)

Laboratory of Molecular Mechanisms of Hematologic Disorders and Therapeutic Implications, Necker Branch, INSERM U1163 and CNRS ERL 8654, Necker-Enfants Malades Hospital (AP-HP), Paris-Centre University, Imagine Institute, Paris, France; CEREMAST, Imagine Institute, INSERM U1163, Paris, France; Department of Hematology, Necker-Enfants Malades Hospital (AP-HP5), Paris-Centre University, Imagine Institute, Paris, France.

Antoine Neuraz (A)

Department of Bioinformatics, Necker Branch, Necker-Enfants Malades Hospital (AP-HP5), Paris-Centre University, Imagine Institute, INSERM U1163, Paris, France.

Djiba Condé (D)

Department of Dermatology, Reference Center for Genodermatoses (MAGEC), Necker-Enfants Malades Hospital (AP-HP5), Paris-Centre University, Imagine Institute, INSERM U1163, Paris, France.

Julie Agopian (J)

CEREMAST, Imagine Institute, INSERM U1163, Paris, France; Centre de Recherche en Cancérologie de Marseille, équipe Labellisée Ligue nationale contre le Cancer, INSERM U1068, Marseille, France; Association Française pour les Initiatives de Recherche sur le Mastocyte et les Mastocytoses (AFIRMM), Marseille, France.

Cécile Méni (C)

Department of Dermatology, Reference Center for Genodermatoses (MAGEC), Necker-Enfants Malades Hospital (AP-HP5), Paris-Centre University, Imagine Institute, INSERM U1163, Paris, France.

Nicolas Garcelon (N)

Department of Bioinformatics, Necker Branch, Necker-Enfants Malades Hospital (AP-HP5), Paris-Centre University, Imagine Institute, INSERM U1163, Paris, France.

Patrice Dubreuil (P)

CEREMAST, Imagine Institute, INSERM U1163, Paris, France; Centre de Recherche en Cancérologie de Marseille, équipe Labellisée Ligue nationale contre le Cancer, INSERM U1068, Marseille, France.

Leïla Maouche-Chrétien (L)

Laboratory of Molecular Mechanisms of Hematologic Disorders and Therapeutic Implications, Necker Branch, INSERM U1163 and CNRS ERL 8654, Necker-Enfants Malades Hospital (AP-HP), Paris-Centre University, Imagine Institute, Paris, France.

Smail Hadj-Rabia (S)

Department of Dermatology, Reference Center for Genodermatoses (MAGEC), Necker-Enfants Malades Hospital (AP-HP5), Paris-Centre University, Imagine Institute, INSERM U1163, Paris, France.

Olivier Hermine (O)

Laboratory of Molecular Mechanisms of Hematologic Disorders and Therapeutic Implications, Necker Branch, INSERM U1163 and CNRS ERL 8654, Necker-Enfants Malades Hospital (AP-HP), Paris-Centre University, Imagine Institute, Paris, France; CEREMAST, Imagine Institute, INSERM U1163, Paris, France; Department of Hematology, Necker-Enfants Malades Hospital (AP-HP5), Paris-Centre University, Imagine Institute, Paris, France.

Christine Bodemer (C)

Department of Dermatology, Reference Center for Genodermatoses (MAGEC), Necker-Enfants Malades Hospital (AP-HP5), Paris-Centre University, Imagine Institute, INSERM U1163, Paris, France. Electronic address: christine.bodemer@aphp.fr.

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Classifications MeSH

questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Criteria for the Regression of Pediatric...
questionsmedicales.fr › Personnes › Tranches d'âge › Enfant › Criteria for the Regression of Pediatric...
questionsmedicales.fr › Environnement et santé publique › Santé publique › Méthodes épidémiologiques › Caractéristiques des études épidémiologiques › Études épidémiologiques › Études de cohortes › Études de suivi › Criteria for the Regression of Pediatric...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Criteria for the Regression of Pediatric...
questionsmedicales.fr › Environnement et santé publique › Santé publique › Méthodes épidémiologiques › Caractéristiques des études épidémiologiques › Études épidémiologiques › Études de cohortes › Études longitudinales › Criteria for the Regression of Pediatric...
questionsmedicales.fr › Systèmes sanguin et immunitaire › Système immunitaire › Mastocytes › Criteria for the Regression of Pediatric...
questionsmedicales.fr › Maladies du système immunitaire › Troubles de l'activation mastocytaire › Mastocytose › Criteria for the Regression of Pediatric...
questionsmedicales.fr › Maladies du système immunitaire › Troubles de l'activation mastocytaire › Mastocytose › Mastocytose généralisée › Criteria for the Regression of Pediatric...
questionsmedicales.fr › Phénomènes génétiques › Variation génétique › Mutation › Criteria for the Regression of Pediatric...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines tumorales › Protéines oncogènes › Protéines proto-oncogènes › Protéines proto-oncogènes c-kit › Criteria for the Regression of Pediatric...