Histiocytární choroby
| Title in English | Histiocytic disorders |
|---|---|
| Authors | |
| Year of publication | 2009 |
| Type | Article in Periodical |
| Magazine / Source | Vnitřní lékařství |
| MU Faculty or unit | |
| Citation | |
| Field | Oncology and hematology |
| Keywords | histioccytosis; Langerhans cell histiocytosis; juvenile xanthogranuloma; Erdheim Chester disease; dinfritic cell sarcoma |
| Description | 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 infiltrace lower in extremities as well as retroperitoneum and mediastinum and are the cause of fibrosis. Both Erdheim-Chester disease and Langerhans cell histiocytosis sometimes infiltrace hypothalamus and pituitary stalk and diabetes insipidus is thus the first sign of the disease. Dendritic cell sarcoma occurs very rarelyand is, unlike the former two disease, resistant to chemotherapy. |
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