Úspěšná léčba Erdheimovy-Chesterovy nemoci chemoterapií obsahující 2-chlorodeoxyadenosin. Popis dvou případů a přehled literatury

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Title in English Successful treatment of Erdheim-Chester disease by 2-chlorodoxyadenosine-based chemotherapy. Two case studies and a literature review.
Authors

ADAM Zdeněk KOUKALOVÁ R. ŠPRLÁKOVÁ-PUKOVÁ Andrea ŘEHÁK Z. ČERVINEK Libor SZTURZ Petr KREJČÍ Marta POUR Luděk ZAHRADOVÁ Lenka MOULIS Mojmír PRÁŠEK Jiří CHALOUPKA Richard HÁJEK Roman MAYER Jiří

Year of publication 2011
Type Article in Periodical
Magazine / Source Vnitřní lékařství
MU Faculty or unit

Faculty of Medicine

Citation
Field Other specializations of internal medicine
Keywords Erdheim-Chester disease; juvenile xanthogranuloma; PET-CT; imagination; bone scan; 2-chlorodeoxyadenosine; cladribine; cyclophosphamide; dexamethasone; fewer of unknown origin; osteosclerosis; hyperostosis; osteoporosis; ertroperitoneal fibrosis
Description Erdheim-Chester disease is an extremely rarely occuring condition and thus an optimaltreatment in not known. Two new cases have been diagnosed in our centre in 2008 and 2009. Both patients had diabetes insipidus, B symptoms (subrefile to febrile states) and pain in long bones of lower limbs. Administration of 2-chlorodeoxyadenosine, cyclophosphamide and dexamethasone led to partial remission in one patient; nearly complete remission of CNS infiltrates but persistent elevation of fluorodeoxyglucose accumulation in bone lesions. Complete remission with significant reduction in accumulation of fluorodeoxyglucose in all disease lesions with normalization of originally increased inflammatory markers and disappearance of all symptoms of the disease was achieved in the second patient.
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