Histiocytic disorders


Authors: Z. Adam;  M. Krejčí;  L. Pour
Authors‘ workplace: Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Vorlíček, CSc.
Published in: Vnitř Lék 2009; 55(Suppl 1)(Supplementum 1): 109-124

Overview

Histiocytic disorders occur sporadically and may thus be considered as rare diseases. The most frequent serious histiocytic disorder of adulthood is the Langerhans cell histiocytosis. Juvenile xanthogranuloma is associated with foamy histiocytes and affects patients in early childhood. Erdheim-Chester disease, on the other hand, is a disease of adulthood. Foamy macrophages usually infiltrate lower extremities as well as retroperitoneum and mediastinum and are the cause of fibrosis. Both Erdheim-Chester disease and Langerhans cell histiocytosis sometimes infiltrate hypothalamus and pituitary stalk and diabetes insipidus is thus the first sign of the disease. Dendritic cell sarcoma occurs very rarely and is, unlike the former two diseases, resistant to chemotherapy.

Key words:
histiocytosis – Langerhans cell histiocytosis – juvenile xanthogranuloma – Erdheim-Chester disease – dendritic cell sarcoma


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