Medica Jadertina, Vol. 39 No. 3-4, 2009.
Professional paper
Mastocytosis in children
Dubravka Kuljiš
; Clinical Hospital Center Split, Clinic of pediatry, Clinical department for hematology, oncology, immunology and genetics
Srđana Čulić
orcid.org/0000-0002-7317-6475
; Clinical Hospital Center Split, Clinic of pediatry, Clinical department for hematology, oncology, immunology and genetics
Višnja Armanda
; Clinical Hospital Center Split, Clinic of pediatry, Clinical department for hematology, oncology, immunology and genetics
Saša Sršen
; Clical Hospital center Split, Clinic of pediatry, Clinical department for hematology, oncology, immunology and genetics
Abstract
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.
Keywords
Hrčak ID:
41973
URI
Publication date:
28.10.2009.
Visits: 9.261 *