Mastocytóza
| Title in English | Mastocytosis |
|---|---|
| Authors | |
| Year of publication | 2015 |
| Type | Chapter of a book |
| MU Faculty or unit | |
| Citation | |
| Description | Mastocytosis represents a heterogeneous group of various disorder characterized by abnormal mast cell proliferation and their accumulation within various organs, most commonly the skin. The World Health Organization (WHO) classification of mastocytosis includes the cutaneous and systematic mastocytosis. The course of the disease ranges fro skin leasions which spontanously regress to aggressive multi-sytem disease with the median of survival of few months. Mast cells are normally derived from pluripotenttial haematopoetic cells and are the effector cells of the immediate allergic reactions. In systemic mastocytosis we usually observe clonal, somatic mutations of c-KIT prot-oncolgen, whose constitutive activation causes mast cell proliferation and suppresion of their apoptosis - clinical symptoms are then due to release of mast cell granules and also to organ infiltration. The therapy consists of symptomatic therapy, the prevention of mediator release. In severe cases of systemic mastocytosis, cytoreduction is indicated, plus many novel drugs such as thalidomid, dasatinib, NF-kappaB inhibitors are currently tested within various clinical trials. However, allogeneic stem cell transplantation remains the only, potentially curative option. |