Mastocytosis is a disease associated with excessive mast cells proliferation. The cause of this disease development is still unclear. Given the rarity, variety of forms, and often benign course, the disease often remains unrecognized. Differences in the course and prognosis of the disease in adults and children make it necessary to view this disease separately. In this article, only mastocytosis in children will be considered. Objective of the research: to present a brief review of literature and epidemiological analysis of the Russian group of children with mastocytosis. Materials and methods: to collect information, parents of children with mastocytosis were questioned through social network. Second contact was preformed through second questionnaire, e-mail, telephone, personal consultation. Results: data were obtained on 111 patients - 54 boys and 57 girls. The median onset of the disease was 2 months (Q1 - 1, Q3 - 4 months). 87% (n=97) patients had urticaria pigmentosa, 9% (n=10) had mastocytoma, and 4% (n=4) had diffuse mastocytosis. The quality of life of most patients generally corresponds to healthy peers. The main complaints (in descending order) are rashes, pruritus, hot flushes, abdominal pain, diarrhea, nausea and vomiting, anaphylactoid reactions. 40% of patients (n=45) do not have systematic therapy, 55% (n=61) of patients regularly take antihistamines, 5% (n=5) of patients regularly take glucocorticoids. With a median follow-up of 37 months (8-137 months), 11,8% of patients noted new symptoms, 58,8% - stabilization and 29,4% - improvement of their condition. Conclusion: child mastocytosis is generally a benign disease, so the main task of the doctor is to select symptomatic therapy and ensure the quality of life of the child.
Translated title of the contributionMastocytosis in children: Characteristic of a group of 111 patients
Original languageRussian
Pages (from-to)135-140
Number of pages6
JournalПЕДИАТРИЯ. ЖУРНАЛ ИМ. Г.Н. СПЕРАНСКОГО
Volume97
Issue number4
DOIs
StatePublished - 2018

    Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

    Research areas

  • Counseling, Mast cell leucosis, Mastocytosis in children, Systemic mastocytosis, Urticaria pigmentosa

ID: 36316775