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Systemic symptoms are typically a result of mast-cell mediator release but do not prove systemic mast-cell hyperplasia.
In this review, we present several research studies related to pediatric mast-cell disorders, and discuss several cases of pediatric mastocytosis, acute myeloid leukemia, pathophysiology, genetic studies, and treatment.
Introduction and Research Studies Pediatric mastocytosis can very easily be mistaken for a variety of common rashes that plague the pediatric population.
Children very commonly present to pediatricians with different rashes ranging from urticaria to eczema to poison ivy. Pediatric mastocytosis is a rare disease, which affects the skin and multiple organs due to an increase in mast-cell load in the body.
Mast cells participate in immune defense and allergic disease. When mast cells degranulate, two of the main enzymes released are histamine and tryptase, which participate in the skin reaction that appears as a rash and is classified as mastocytosis.
There has, however, been no gender bias noted in the cases of pediatric mastocytosis. The three major types of cutaneous mastocytosis are urticaria pigmentosa UP, diffuse cutaneous mastocytosis DCM, and solitary mastocytoma. Mast cells degranulate in various organ systems other than the skin, and this is classified as systemic mastocytosis.
Mast-Cell Hyperplasia In order to analyze the extent of mast-cell hyperplasia, core bone-marrow biopsies were performed and analyzed. In one study, it was found that in 10 of 17 children with mastocytosis, there were focal areas of mast-cell hyperplasia, which had perivascular and paratrabecular aggregates of mast cells, eosinophils, and early myeloid cells.
Role of Histamine Histamine is the major enzyme released during mast-cell degranulation. Normal plasma levels of histamine range from about 0. It was found that histamine levels were increased in DCM, and were seven times the normal amount in UP.
Similar to histamine-level findings, it was found that tryptase levels in patients with DCM were higher than tryptase levels found in patients with UP.
The total tryptase levels are highest in young infants at about 6.
It was found that the tryptase levels in the 72 atopic and allergic infants was highest at 14. This method provides standardized information on the severity of cutaneous mastocytosis and imposes no burden on the patient, which is particularly important in children.
In patients with UP, a tryptase concentration of 7. Patients with mastocytoma had a tryptase level of 4.