Abstract
Systemic mastocytosis (SM) is a rare and acquired myeloproliferative disorder characterized by a clonal proliferation of neoplastic mast cells and their accumulation in tissues. In contrast to purely cutaneous form is systemic mastocytosis a disease of adulthood.
The symptoms of the disease are partly related to the organ infiltration, partly are caused by the release of mediators from neoplastic mast cells. The most important diagnostic tools are histology with immunohistochemistry of the infiltrated organ (particularly bone marrow), molecular analyses and the assesment of serum tryptase level.