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Mastocytosis in Children.

Nicholas Klaiber1, Santhosh Kumar1, Anne-Marie Irani2

  • 1Department of Pediatrics, Division of Allergy and Immunology, Virginia Commonwealth University Health Systems, 1000 E Broad Street, Richmond, VA, 23219, USA.

Current Allergy and Asthma Reports
|October 9, 2017
PubMed
Summary

Pediatric mastocytosis typically presents as skin lesions with a good prognosis and often resolves by adolescence. However, systemic mastocytosis is more severe and may continue into adulthood.

Keywords:
Mast cellMast cell activationMastocytosisPediatric mastocytosisPediatric mastocytosis prognosisTryptase

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Area of Science:

  • Pediatric Hematology
  • Dermatology
  • Immunology

Background:

  • Mastocytosis is a rare disorder characterized by the abnormal proliferation of mast cells.
  • Understanding its varied presentations and natural history in children is crucial for effective management.

Purpose of the Study:

  • To review the current knowledge on pediatric mastocytosis, including its pathogenesis, clinical features, diagnosis, and treatment.
  • To elucidate the natural history and prognosis of childhood mastocytosis.

Main Methods:

  • Comprehensive literature review of pediatric mastocytosis.
  • Analysis of pathogenesis, clinical manifestations, diagnostic approaches, and therapeutic strategies.
  • Evaluation of disease progression and long-term outcomes.

Main Results:

  • Pediatric mastocytosis commonly manifests as cutaneous lesions with a favorable prognosis and frequent spontaneous remission by adolescence.
  • Systemic mastocytosis involving organ systems is a rarer, more severe form with a tendency to persist into adulthood.
  • Emerging concepts like mast cell activation syndrome are also considered.

Conclusions:

  • Early identification and appropriate management are key for pediatric mastocytosis.
  • Distinguishing between cutaneous and systemic forms is vital for predicting disease course and prognosis.
  • Further research into mast cell activation syndrome may offer new therapeutic avenues.