[Mastocytosis--diagnostic criteria and treatment].
Mastocytosis is a heterogeneous group of rare diseases characterized by the proliferation and accumulation of mast cells in one or more organs such as the skin, bone marrow, liver, spleen, and lymph nodes. According to the WHO classification, mastocytosis is divided into seven subvariants. The symptoms are associated with mediator release and impaired organ function due to infiltration by neoplastic mast cells. There is a higher risk of anaphylactic shock; therefore education of the patients is very important. Patients may be asymptomatic. Symptomatic treatment is used in cutaneous mastocytosis and in indolent systemic mastocytosis. More aggressive subvariants of mastocytosis are treated with chemotherapy, targeted therapy, and bone marrow transplantation.
Dereń-Wagemann
Izabela
I
Klinika Hematologii, Nowotworów Krwi i Transplantacji Szpiku, Samodzielny Publiczny Szpital Kliniczny nr 1 we Wrocławiu. izabeladw@gmail.com
Kuliszkiewicz-Janus
Małgorzata
M
Kuliczkowski
Kazimierz
K
D003937:Diagnosis, Differential; D006801:Humans; D007154:Immune System Diseases / Q000145:classification / Q000175:diagnosis / Q000628:therapy; D008407:Mast Cells / Q000276:immunology*; D008415:Mastocytosis / Q000145:classification / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy*; D011379:Prognosis; D035583:Rare Diseases; D012867:Skin / Q000276:immunology; D014582:Urticaria Pigmentosa / Q000145:classification / Q000175:diagnosis / Q000628:therapy; D014944:World Health Organization