Histiocytoses are rare, often systemic diseases hallmarked by tissue infiltration by abnormal histiocytes bearing peculiar morphological and immunohistochemical characteristics. They are classified based on cell type (e.g. Langerhans cell histiocytosis (LCH) and non-Langerhans forms such as Erdheim-Chester disease (ECD)), preferential organ involvement (e.g. cutaneous forms) and clinical aggressiveness (e.g. malignant histiocytoses). Some histiocytoses are clearly clonal disorders, often driven by somatic mutations of proto-oncogenes, whereas others can be reactive to or associated with systemic immune-mediated or infectious diseases. This chapter will mainly focus on primary systemic histiocytoses such as LCH and ECD and will discuss the role of infections in these complex diseases.
Histiocytoses / Vaglio A.; Rocco R.; Haroche J.; Emile J.-F.. - ELETTRONICO. - (2018), pp. 379-390. [10.1007/978-3-319-79026-8_29]
Histiocytoses
Vaglio A.;
2018
Abstract
Histiocytoses are rare, often systemic diseases hallmarked by tissue infiltration by abnormal histiocytes bearing peculiar morphological and immunohistochemical characteristics. They are classified based on cell type (e.g. Langerhans cell histiocytosis (LCH) and non-Langerhans forms such as Erdheim-Chester disease (ECD)), preferential organ involvement (e.g. cutaneous forms) and clinical aggressiveness (e.g. malignant histiocytoses). Some histiocytoses are clearly clonal disorders, often driven by somatic mutations of proto-oncogenes, whereas others can be reactive to or associated with systemic immune-mediated or infectious diseases. This chapter will mainly focus on primary systemic histiocytoses such as LCH and ECD and will discuss the role of infections in these complex diseases.
I documenti in FLORE sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
