Onkologie. 2016:10(5):206-209 | DOI: 10.36290/xon.2016.044

Histiocytoses in kids and young adults

Zdenka Křenová, Viera Bajčiová, Jaroslav Štěrba

Klinika dětské onkologie a hematologie FN Brno

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

skeleton, 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. Patients

with MS-LCH without involvement of “risk organs” have very high (>95%) probability of survival when treated with a standard

regimen consisting of vinblastine and steroids. In contrast, involvement of risk organs carries the risk of unfavorable outcome.

Patients with reactivations or chronic disease may experience severe permanent consequences (PC) reducing the patient’s quality

of life, in particular when they affect the CNS or lungs and lead to hormone deficiencies, a neurodegenerative syndrome, lung

fibrosis, etc. The rare histiocytic disorders (RHD), or non-Langerhans cell disorders, is a diverse group of disorders defined by the

accumulation of histiocytes that do not meet the criteria for Langerhans cell histiocytosis (LCH) or hemophagocytic lymphohistiocytosis

(HLH). They include: juvenile xanthogranuloma family, Erdheim-Chester disease, multifocal reticulohistiocytosis,

Rosai-Dorfman disease and the malignant histiocytoses.

Keywords: LCH, non-LCH, BRAF-V600E mutace, activated ERK pathway

Published: November 1, 2016  Show citation