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Pediatric Mastocytosis

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Mastocytosis

Abstract

Patients of all ages are diagnosed with mastocytosis, and in children, the manifestations primarily involve the skin. Although children are mainly diagnosed with cutaneous mastocytosis, they can experience generalized symptoms involving other organ systems such as the GI tract, neurologic, and musculoskeletal systems. Most patients with systemic disease have a somatically acquired activating mutation in the KIT oncogene. Pediatric-onset disease differs from adult-onset disease by variant, disease manifestations, therapy, and prognosis to include resolution. This chapter encapsulates the disease spectrum of mastocytosis in children, with an overview of the clinical features and the approach to diagnosis, evaluation, management, and prognosis.

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Abbreviations

BM:

Bone marrow

CM:

Cutaneous mastocytosis

DCM:

Diffuse cutaneous mastocytosis

ISM:

Indolent systemic mastocytosis

MC:

Mast cell

MIS:

Mastocytosis in the skin

MPCM:

Maculopapular cutaneous mastocytosis

NIH:

National Institutes of Health

PB:

Peripheral blood

qPCR:

quantitative polymerase chain reaction

REMA:

Spanish Network on Mastocytosis

SM:

Systemic mastocytosis

UP:

Urticaria pigmentosa

WHO:

World Health Organization

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Author information

Authors and Affiliations

  1. Instituto de Estudios de Mastocitosis de Castilla-La Mancha (CLMast), Hospital Virgen del Valle, Toledo, Spain

    Iván Alvarez-Twose

  2. The Spanish Network on Mastocytosis (REMA), Toledo and Salamanca, Spain

    Iván Alvarez-Twose

  3. Mast Cell Biology Section, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA

    Melody C. Carter

Authors
  1. Iván Alvarez-Twose
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  2. Melody C. Carter
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Correspondence to Melody C. Carter .

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Editors and Affiliations

  1. Professor of Medicine, Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, MI, USA

    Cem Akin

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Alvarez-Twose, I., Carter, M.C. (2020). Pediatric Mastocytosis. In: Akin, C. (eds) Mastocytosis. Springer, Cham. https://doi.org/10.1007/978-3-030-27820-5_6

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