Publication details

Klinické obrazy histiocytózy z Langerhansových buněk v dospělosti.

Authors

ADAM Zdeněk JEŽOVÁ Marta NEBESKÝ T ŘEHÁK Z FASSMAN A SMILEK P KREJČÍ Marta POUR Luděk KRÁL Zdeněk

Year of publication 2019
Type Article in Periodical
Magazine / Source Transfúze a hematologie dnes
Citation
Keywords Langerhans cell histiocytosis
Description Langerhans cell histiocytosis (LCH) is a rare disease affecting both genders, which may occur at any age. It often involves recurrent flare-ups and its severity varies from benign forms that do not require treatment to life threatening disease. Some patients have serious functional impairment with psychological and social consequences and prolonged disability. LCH may affect a single organ, with uni- or multifocal involvement or it can involve multiple organs -multisystemic disease. The organs most frequently involved are bones, lung, skin and the endocrine system. Isolated pulmonary LCH is related to smoking, but this is not true in the case of multisystemic disease. Some patients have mixed histiocytosis combining LCH and other histiocytic disorders. The diagnosis is based on histological examination of tissue samples that demonstrates tissue infiltration with cells staining for CD1a and Langerin (CD207) on immunohistochemistry. The BRAFV600E mutation is observed in tissue samples in approximately one half of patients. Treatment must be adapted to the severity of the disease and ranges from conservative observation to systemic chemotherapy. Therapies targeting the RAS-RAF-MEK-ERK pathway are promising treatments for progressive disease in patients treated with 2-chlorodeoxyadenosine or vinca alkaloids and glucocorticoids.