|Hanna Ługowska-Umer, Magdalena Lange, Monika Zabłotna, Jolanta Gleń, Joanna Chylicka, Monika Sikorska, Bogusław Nedoszytko, Roman Nowicki|
Introduction. Mastocytosis belongs to group of myeloproliferative diseases characterized by abnormal activation and accumulation of mast cells (MC) in internal organs and skin. Pediatric type of mastocytosis is usually confined to the skin. The activation of MC causes the release of MC mediators, what can provoke several clinical symptoms.
Aim. Evaluation of frequency of mast cell mediator-related symptoms among children with cutaneous mastocytosis.
Material and methods. The clinical symptoms were analyzed in 180 children (100 boys, 80 girls) with diagnosis of mastocytosis. In subjects laboratory tests, including determination of serum tryptase levels were performed.
Results. In the group of 180 children, 83.8% presented the maculopapular cutaneous mastocytosis (MPCM) type of cutaneous mastocytosis (CM), 10% mastocytoma, and 6.1% were diagnosed with diffuse cutaneous mastocytosis (DCM). Among the mast cell mediator-related symptoms, the most common were: pruritus (57.2%), flushing (30%), blistering lesions (27.8%). Those symptoms appeared in all patients with DCM. Mast cell mediator-related symptoms appeared most frequently in children with serum tryptase levels above 20 ng/ml. Anaphylactic reactions appeared also more frequently in children with serum tryptase levels above 20 ng/ml (27.8% cases), while in children with the level 0 ng/ml to 11 ng/ml, those reactions appeared in 3.4% cases.
Conclusions. The most severe type of CM is DCM, not only because of the extent of skin lesions but also because of more frequent mast cell mediator-related symptoms. Serum tryptase level above 20 ng/ml is associated with higher risk of mast cell mediator-related symptoms.
keywords: mastocytoza skórna, dzieci, tryptaza, objawy, skin mastocytosis, children, tryptase, symptoms
pages: from 235 to 240
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