Ján Lazúr
Mastocytosis is a group of clonal disorders, that are characterised by abnormal expansion and accumulation of neoplastic
mast cells. Clinical manifestations are very heterogenous, from isolated skin form, mostly in children, to serious
stages os systemic mastocytosis, occuring in adults. According to WHO classification, there is indolent SM (ISM) with
symptoms caused predominantly by degranulation of mast cells, futhermore, aggressive form of SM (ASM), SM associated
with hematological neoplasia (SM-AHN) and mast cell leukemia (MCL), characterised by by organ dysfunction
caused by infiltration by mast cells. Basic diagnostic step is biopsy of skin and bone marrow, and histological finding of
multifocal clusters of morphologically abnormal mast cells is pathognomic for this diagnosis. Therapy is paliative, focused
on lowering the symptoms caused by mediators from activated mast cells. In advanced stages of SM, a cytoreduction
may be used.