Absence of circulating mast cell precursors in paediatric mastocytosis: could it reflect a different pathophysiology between adults and children with mastocytosis?

Authors

  • S. Georgin-Lavialle,

    1. CNRS UMR 8147, Hôpital Necker-Enfants malades, Paris, France
    2. Centre de Référence des Mastocytoses, Faculté de Médecine et AP-HP Necker-Enfants Malades, Paris, France
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    • These authors corresponded equally to the work.
  • L. Le Saché-de Peufeilhoux,

    1. Service de Dermatologie, Université Paris Descartes, Paris Sorbonne Cité, Faculté de Médecine et AP-HP Necker-Enfants Malades, Paris, France
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    • These authors corresponded equally to the work.
  • L. Martin,

    1. Service de dermatologie, Université UNAM, hôpital d'Angers, Angers, France
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  • E. Soucie,

    1. Inserm, U1068, CRCM,[ Signaling, Hematopoiesis and Mechanism of Oncogenesis]; Institut Paoli-Calmettes, Marseille; Aix-Marseille Univ; CNRS, UMR7258,CRCM, Marseille, France
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  • J. Bruneau,

    1. Laboratoire d'anatomopathologie, Université Paris Descartes, Paris Sorbonne Cité, Faculté de Médecine et AP-HP Necker-Enfants Malades, Paris, France
    2. Institut Image, Necker, Paris, France
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  • S. Barete,

    1. CNRS UMR 8147, Hôpital Necker-Enfants malades, Paris, France
    2. Centre de Référence des Mastocytoses, Faculté de Médecine et AP-HP Necker-Enfants Malades, Paris, France
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  • P. Dubreuil,

    1. Centre de Référence des Mastocytoses, Faculté de Médecine et AP-HP Necker-Enfants Malades, Paris, France
    2. Inserm, U1068, CRCM,[ Signaling, Hematopoiesis and Mechanism of Oncogenesis]; Institut Paoli-Calmettes, Marseille; Aix-Marseille Univ; CNRS, UMR7258,CRCM, Marseille, France
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  • C. Bodemer,

    1. Centre de Référence des Mastocytoses, Faculté de Médecine et AP-HP Necker-Enfants Malades, Paris, France
    2. Service de Dermatologie, Université Paris Descartes, Paris Sorbonne Cité, Faculté de Médecine et AP-HP Necker-Enfants Malades, Paris, France
    3. Institut Image, Necker, Paris, France
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  • O. Hermine,

    Corresponding author
    1. CNRS UMR 8147, Hôpital Necker-Enfants malades, Paris, France
    2. Centre de Référence des Mastocytoses, Faculté de Médecine et AP-HP Necker-Enfants Malades, Paris, France
    3. Service d'Hématologie Adultes, Université Paris Descartes, Paris Sorbonne Cité, Faculté de Médecine et AP-HP Necker-Enfants Malades, Paris, France
    4. Institut Image, Necker, Paris, France
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    • Both authors co-directed this work.
  • L. Lhermitte

    1. CNRS UMR 8147, Hôpital Necker-Enfants malades, Paris, France
    2. Laboratoire d'hématologie Biologique et UMR CNRS 8147, Université Paris Descartes, Paris Sorbonne Cité, Faculté de Médecine et AP-HP Necker-Enfants Malades, Paris, France
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    • Both authors co-directed this work.

  • Conflict of interest

      Conflict of interest
    • The authors declare no conflict of interest.
  • Funding sources

      Funding sources
    • Sophie Georgin-Lavialle is a recipient of a grant from SNFMI-Genzyme-maladies rares and Centre National de la Recherche Scientifique (CNRS) and Assistance Publique Hôpitaux de Paris (AP-HP).

Abstract

Background

Mastocytosis is a heterogeneous disease whose different subtypes also vary in aggressivity. Children typically present with cutaneous mastocytosis. We identified, in our previous work, a peripheral CD34-c-Kit+mast cell precursor by flow cytometry in systemic forms but not in cutaneous forms of adult mastocytosis.

Objectives

We wanted to know if such a mast cell precursor exists among children with mastocytosis.

Methods

We analysed 10 children with mastocytosis for c-Kit+CD34- mast cell precursors by flow cytometry.

Results

In contrast to adults with mastocytosis, we did not detect any circulating mast cell precursors by flow cytometry in the peripheral blood of children with mastocytosis.

Conclusion

The clinical symptoms observed among children with cutaneous mastocytosis could be induced by cutaneous mast cell mediators and not by circulating mast cells. These results may help to better understand the differences between adult and childhood mastocytosis.

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