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Pediatric mastocytosis
1Division of Allergy and Clinical Immunology, Children's Hospital Medical Center, Elland and Bethesda Avenue, Cincinnati, Ohio, USA.
Insights
Pediatric mastocytosis often presents with skin issues like urticaria pigmentosa, differing from adult cases. While internal organ involvement is less common in children, some may see symptoms resolve by adolescence.
Area of Science:
- Pediatric Hematology
- Dermatology
- Allergy and Immunology
Background:
- Mastocytosis is a rare disorder characterized by the abnormal accumulation of mast cells.
- Pediatric-onset mastocytosis accounts for a significant portion of all cases, with onset typically occurring in early childhood.
Purpose of the Study:
- To describe the distinct characteristics of pediatric-onset mastocytosis.
- To outline the typical presentations, potential organ involvement, and treatment strategies for children with mastocytosis.
Main Methods:
- Review of clinical presentations and disease progression in pediatric patients.
- Analysis of common symptoms, affected organs, and therapeutic interventions.
Main Results:
- Pediatric mastocytosis commonly manifests as cutaneous lesions such as solitary mastocytoma or urticaria pigmentosa.
- Internal organ involvement, including bone marrow and gastrointestinal tract, is less frequent in children compared to adults.
- Elevated plasma histamine levels can be observed in pediatric cases.
Conclusions:
- Pediatric mastocytosis presents differently from adult-onset forms, primarily with skin manifestations.
- Treatment often involves antihistamines for symptom management.
- Prognosis varies, with a notable percentage of children experiencing symptom resolution by adolescence.
Abstract:
The onset of mastocytosis occurs between birth and 2 years of age in approximately 55% of all cases; an additional 10% develop the disease before the age of 15 years. Mastocytosis in these age groups differs in many respects from mastocytosis that has its onset in adulthood. The typical presentation of pediatric-onset mastocytosis consists of cutaneous manifestations: either a solitary mastocytoma, urticaria pigmentosa, or, less commonly, diffuse cutaneous mastocytosis. Particularly in infants, bullous eruptions may occur. Mastocytosis in infants and children may involve internal organs, including the bone marrow and the gastrointestinal tract, although such manifestations appear to be less common in children than in adults. Plasma histamine levels may be elevated in pediatric-onset mastocytosis. Treatment usually involves the use of H1 and H2 antihistamines to control itching and to control the hypersecretion of gastric acid that may occur. The prognosis for children with mast cell disease is variable; approximately half of the children with urticaria pigmentosa may experience resolution of lesions and symptoms by adolescence.