Mastocytosis in children

Mastocitoza je rijetka bolest karakterizirana povecanim stvaranjem i nakupljanjem tkivnih mastocita. Klinicka slika ovisi o mjestu nakupljanja mastocita, o degranulaciji mastocita i oslobadanju medijatora. Urtikarija, crveno-smede makule, papule i plakovi mogu se javiti po citavom tijelu, narocito na trupu. Mastocitoza u djecjoj dobi je najcešce benigna bolest prezentirana nakupljanjem mastocita u koži i ne zahtijeva terapijsku intervenciju. Ucestalost mastocitoze u našoj klinickoj patologiji je 0,06%. Analizirali smo simptome i klinicku sliku kod 10 bolesnika, kod kojih je klinicki i patohistološki dokazana mastocitoza. Kod svih 10 bolesnika, osam djecaka i dvije djevojcice, naden je samo kožni oblik bolesti bez zahvacenosti organa ili sistemnih znakova oslobadanja medijatora iz mastocita. Dijagnoza je postavljena u dojenackom razdoblju, a prosjecna dob bila je 3,5 mjeseca (raspon 0-9 mjeseci). Nalaz koštane srži, biokemijske i ultrazvucne pretrage parenhimnih organa bile su uredne. Deset godišnje pracenje naših bolesnika pokazalo je da su simptomi bolesti kod svih bolesnika polako i spontano nestajali, te djeca nisu imala potrebe za lijecenjem.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