Publication details
Hodnocení pětileté léčby Erdheimovy-Chesterovy nemoci anakinrou – kazuistika a přehled literatury
| Title in English | Evaluation of five years of treatment of Erdheim-Chester disease with anakinra: case report and overview of literature |
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| Authors | |
| Year of publication | 2016 |
| Type | Article in Periodical |
| Magazine / Source | Vnitřní lékařství |
| MU Faculty or unit | |
| Citation | |
| Field | Oncology and hematology |
| Keywords | anakinra; Erdheim-Chester disease |
| Description | Erdheim-Chester disease is a histiocytic neoplasm of diseases from the group of non-Langerhans-cell histiocyto-ses, formed by infiltrates of foamy histiocytes. These pathological histiocytes produce pro-inflammatory cytokines. Therefore Erdheim-Chester disease is called inflammatory histiocytary neoplasm. The disease is accompanied by clinical symptoms of systemic inflammatory response, i.e. B symptoms. Imaging examinations detect typical osteo-sclerotic changes affecting diaphyses and metaphyses of the lower long bones and fibrotic changes which affect the aorta wall and the vessels leading from it. Also characteristic are perirenal fibrotic changes spreading in the retroperitoneum. They can cause serious complications - hydronephrosis with all its consequences. |
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