Histiocytózy u dětí a dospívajících

Title in English Histiocytoses in kids and young adults


Year of publication 2016
Type Article in Periodical
Magazine / Source Onkologie
MU Faculty or unit

Faculty of Medicine

Field Oncology and hematology
Keywords LCH; non-LCH; BRAF-V600E mutace; activated ERK pathway
Description Histiocytoses (LCH and non-LCH) are rare diseases caused by an excess of cells called histiocytes, which can infiltrate the skin, bones, lungs, liver, spleen and the central nervous system. These disorders can range from localized involvement that resolves spontaneously, to progressive disseminated forms that can be debilitating and sometimes life-threatening. There are two widely recognized categories for histiocytoses: LCH and non LCH. According to disease extent we divide LCH to single-system LCH (involvement of a single organ or system) and multisystem LCH (involvement of 2 or more organ systems). Patients with SS-LCH of the :- e etcn, skin, or the lymph nodes have an excellent prognosis and are felt to need a minimum or sometimes even no treatment at all. Recent large clinical trials have shown that the response to initial treatment is a highly important prognostic factor.

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