Stručni rad

Mastocytosis in children

Dubravka Kuljiš ; KBC Split, Klinika za dječje bolesti, Klinički odjel za hematologiju, onkologiju, imunologiju i medicinsku genetiku, (Dubravka Kuljiš, dr.med.)
Srđana Čulić orcid.org/0000-0002-7317-6475 ; KBC Split, Klinika za dječje bolesti, Klinički odjel za hematologiju, onkologiju, imunologiju i medicinsku genetiku, (doc.dr.sc. Srđana Čulić, dr.med.)
Višnja Armanda ; KBC Split, Klinika za pedijatriju, Klinički odjel za hematologiju, onkologiju, imunologiju i medicinsku genetiku, (Višnja Armanda, dr.med.)
Saša Sršen ; KBC Split, Klinika za pedijatriju, Klinički odjel za hematologiju, onkologiju, imunologiju i medicinsku genetiku

Sažetak

Mastocytosis is a rare disease characterised by overproliferation and accumulation of tissue mast cells.
The clinical signs depend on local accumulation of mast cells in different organs and also on the effects of mast cell degranulation and release of mast cell mediators. Mastocytosis in childhood is usually an indolent disease and presented as cutaneous mastocytosis, and does not need therapeutic intervention. Urticaria,
reddish-brown macules, papules and plaques may manifest over the whole body especially on the trunk. The frequency of mastocytosis in our internal pathology is 0,06%. We have analysed the symptoms and clinical course in 10 children with mastocytosis. All analyzed patients, 8 boys and 2 girls, had only indolent cutaneous mastocytosis without extracutaneous involvement or systemic release of mast-cell mediators. All of them had clinical and pathohistological evidence of mast cell proliferation. At the time of diagnosis our patients were infants, mean age of 3,5 months (range 0-9 months). The results of bone marrow analysis were normal, the same as the biochemical and ultrasound examination of parenchymal organs. A ten-year followup revelaed that the disease showed slow spontaneous regression and there was no need for therapy.

Ključne riječi

mastocytosis; children

Hrčak ID:

41973

URI

https://hrcak.srce.hr/41973

Datum izdavanja:

28.10.2009.

Podaci na drugim jezicima: hrvatski

Posjeta: 9.089 *