Mastocytozy u dzieci i dorosłych – rozpoznawanie i postępowanie

*Dorota Czelej, Janusz Urban, Dorota Wojnowska, Aldona Pietrzak, Grażyna Chodorowska, Bartłomiej Wawrzycki, Joanna Bartosińska

Streszczenie
Mastocytosis are heterogenous group of diseases, characterised by infiltrations of mast cells in different tissues. Diagnosis can be made on the basis of Darier´s sign and typical histological findings. In childhood mild – course disease is most often, usually involving only the skin (cutaneous mastocytosis) and show spontaneous remission after a few years. In adults systemic mastocytosis occur more freuquently with other tissues involvement, particurarly bone marrow. Rarely, mastocytosis with neoplastic proliferation of mast cell line or other bone marrow cell lines, appear. Punctate mutations on SCF receptor are often observed, especially in systemic disease. In mild-course mastocytosis drugs, that are able to prevent mastoscytes´ degranulation are administered and antihistaminics, that decrease symptoms of the disease. Very important profilactis is avoidance of histaminoliberatotrs. Patients with agressive forms of disease are treated on Haematological Departments.

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