. . . "I, P(ED2.1.00/03.0101)" . . . "http://www.vnitrnilekarstvi.eu/vnitrni-lekarstvi-clanek/pet-ct-dokumentovana-kompletni-4leta-remise-erdheimovy-chesterovy-nemoci-po-lecbe-kladribinem-48984?confirm_rules=1" . . "PET-CT dokumentovan\u00E1 kompletn\u00ED remise Erdheimovy-Chesterovy nemoci, trvaj\u00EDc\u00ED ji\u017E \u010Dty\u0159i roky od zah\u00E1jen\u00ED l\u00E9\u010Dby kladribinem"@cs . . "CZ - \u010Cesk\u00E1 republika" . . "PET-CT documented complete remission of Erdheim-Chester disease, lasting more than 4 years from treatment initiation with cladribine"@en . "Erdheim-Chester disease is a very rare histiocytic disease. It represents one form of juvenile xanthogranuloma in WHO classification of blood diseases. The disease often causes B symptoms, skeletal pain and also may cause diabetes insipidus and retroperitoneal fibrosis. Selection of therapy depends on published case reports and small clinical trials. There are no recommendations for treatment based on randomized studies. Interferon \u03B1 is probably the most commonly used drug for this disease. Some remissions have been described after treatment. However, long-term interferon \u03B1 application is needed which is associated with numerous side effects. There are limited experiences with clabridine in this indication. Based on the 10 publications we choose cladribine as first-line treatment in our patient. The treatment started in October 2009 with combination of 2-chlorodeoxyadenosine (Litak) 5 mg/m2 sc. + cyclophosphamide 150 mg/m2 iv. + dexamethasone 24 mg iv., five days consecutively. These cycles were repeated monthly. Mentioned formula was submitted 4 times and 3 times in limited application on day 1 - 3. The reason of that was neutropenia grade 3. All symptoms disappeared after treatment. Only diabetes insipidus persisted because damage of pituitary stalk is irreversible. PET-CT imaging showed complete remission of disease and 4.5 years duration of remission after treatment. The treatment was well tolerated with no complications implying hospitalization. Only mild thrombocytopenia and neutropenia remains after 4.5 years. Based on case report and publications we consider cladribine as appropriate firs-line drug for Erdheim-Chester disease."@en . "13"^^ . "60" . "[AD990E10AEFC]" . . . "anakinra; cladribine; Erdheim-Chester disease; interferon \u03B1; juvenile xanthogranuloma; PET-CT in diagnosis of fever of unknown origin; verumafenib 2; chlorodeoxydenosin"@en . "\u0158eh\u00E1k, Zden\u011Bk" . . . "PET-CT dokumentovan\u00E1 kompletn\u00ED remise Erdheimovy-Chesterovy nemoci, trvaj\u00EDc\u00ED ji\u017E \u010Dty\u0159i roky od zah\u00E1jen\u00ED l\u00E9\u010Dby kladribinem"@cs . . . "Erdheimova-Chesterova nemoc je onemocn\u011Bn\u00ED pat\u0159\u00EDc\u00ED mezi velmi vz\u00E1cn\u00E9 histiocyt\u00E1rn\u00ED choroby. P\u0159edstavuje jednu z forem chorob, \u0159azen\u00FDch posledn\u00ED WHO klasifikac\u00ED krevn\u00EDch chorob do skupiny juveniln\u00EDho xantogranulomu. Nemoc \u010Dasto p\u016Fsob\u00ED B symptomy, d\u00E1le bolesti skeletu a m\u016F\u017Ee zp\u016Fsobit i diabetes insipidus i fibr\u00F3zu retroperitonea. Pro tuto nemoc neexistuje l\u00E9\u010Debn\u00E9 doporu\u010Den\u00ED zalo\u017Een\u00E9 na randomizovan\u00FDch studi\u00EDch. Interferon \u03B1 je z\u0159ejm\u011B nej\u010Dast\u011Bji pou\u017E\u00EDvan\u00FD l\u00E9k pro tuto nemoc, byly po n\u011Bm pops\u00E1ny remise. Je v\u0161ak nutn\u00E1 dlouhodob\u00E1 aplikace interferonu \u03B1 a ta je spojena s \u010Detn\u00FDmi ne\u017E\u00E1douc\u00EDmi \u00FA\u010Dinky. Zku\u0161enost\u00ED s kladribinem v t\u00E9to indikaci je o n\u011Bco m\u00E9n\u011B. Na z\u00E1klad\u011B 10 publikac\u00ED jsme zvolili pro popisovan\u00E9ho pacienta kladribin jako l\u00E9\u010Dbu prvn\u00ED linie. L\u00E9\u010Dba byla zah\u00E1jena v \u0159\u00EDjnu roku 2009 kombinac\u00ED 2-chlorodeoxyadenozin (Litac) 5 mg/m2 s. c. + cyklofosfamid 150 mg/m2 i. v. + dexametazon 24 mg i.v., v\u0161e 5 dn\u00ED po sob\u011B. Opakov\u00E1n\u00ED t\u011Bchto cykl\u016F bylo pl\u00E1nov\u00E1no v m\u011Bs\u00ED\u010Dn\u00EDch intervalech. Chemoterapii jsme podali 4kr\u00E1t ve v\u00FD\u0161e uveden\u00E9m slo\u017Een\u00ED a 3kr\u00E1t s aplikac\u00ED omezenou pouze na 1.-3. den. D\u016Fvodem pro redukci byla neutropenie grade III. Po ukon\u010Den\u00ED l\u00E9\u010Dby vymizely v\u0161echny symptomy nemoci, p\u0159etrv\u00E1v\u00E1 pouze diabetes insipidus, proto\u017Ee po\u0161kozen\u00ED stopky hypof\u00FDzy touto chorobou je ireverzibiln\u00ED. PET-CT prok\u00E1zalo dosa\u017Een\u00ED kompletn\u00ED remise nemoci a trv\u00E1n\u00ED t\u00E9to remise po 4,5 letech od zah\u00E1jen\u00ED l\u00E9\u010Dby. L\u00E9\u010Dba byla dob\u0159e tolerov\u00E1na, v pr\u016Fb\u011Bhu l\u00E9\u010Dby se nevyskytly \u017E\u00E1dn\u00E9 komplikace, vynucuj\u00EDc\u00ED si hospitalizaci. Po 4,5 letech od ukon\u010Den\u00ED l\u00E9\u010Dby p\u0159etrv\u00E1v\u00E1 m\u00EDrn\u00E1 trombocytopenie a neutropenie. Na z\u00E1klad\u011B popsan\u00E9ho p\u0159\u00EDpadu a citovan\u00E9 literatury pova\u017Eujeme kladribin za vhodn\u00FD l\u00E9k prvn\u00ED linie pro l\u00E9\u010Dbu Erdheimovy-Chesterovy choroby."@cs . "2"^^ . . "12"^^ . "PET-CT dokumentovan\u00E1 kompletn\u00ED remise Erdheimovy-Chesterovy nemoci, trvaj\u00EDc\u00ED ji\u017E \u010Dty\u0159i roky od zah\u00E1jen\u00ED l\u00E9\u010Dby kladribinem" . . . "Vnit\u0159n\u00ED l\u00E9ka\u0159stv\u00ED" . "PET-CT documented complete remission of Erdheim-Chester disease, lasting more than 4 years from treatment initiation with cladribine"@en . . "Erdheimova-Chesterova nemoc je onemocn\u011Bn\u00ED pat\u0159\u00EDc\u00ED mezi velmi vz\u00E1cn\u00E9 histiocyt\u00E1rn\u00ED choroby. P\u0159edstavuje jednu z forem chorob, \u0159azen\u00FDch posledn\u00ED WHO klasifikac\u00ED krevn\u00EDch chorob do skupiny juveniln\u00EDho xantogranulomu. Nemoc \u010Dasto p\u016Fsob\u00ED B symptomy, d\u00E1le bolesti skeletu a m\u016F\u017Ee zp\u016Fsobit i diabetes insipidus i fibr\u00F3zu retroperitonea. Pro tuto nemoc neexistuje l\u00E9\u010Debn\u00E9 doporu\u010Den\u00ED zalo\u017Een\u00E9 na randomizovan\u00FDch studi\u00EDch. Interferon \u03B1 je z\u0159ejm\u011B nej\u010Dast\u011Bji pou\u017E\u00EDvan\u00FD l\u00E9k pro tuto nemoc, byly po n\u011Bm pops\u00E1ny remise. Je v\u0161ak nutn\u00E1 dlouhodob\u00E1 aplikace interferonu \u03B1 a ta je spojena s \u010Detn\u00FDmi ne\u017E\u00E1douc\u00EDmi \u00FA\u010Dinky. Zku\u0161enost\u00ED s kladribinem v t\u00E9to indikaci je o n\u011Bco m\u00E9n\u011B. Na z\u00E1klad\u011B 10 publikac\u00ED jsme zvolili pro popisovan\u00E9ho pacienta kladribin jako l\u00E9\u010Dbu prvn\u00ED linie. L\u00E9\u010Dba byla zah\u00E1jena v \u0159\u00EDjnu roku 2009 kombinac\u00ED 2-chlorodeoxyadenozin (Litac) 5 mg/m2 s. c. + cyklofosfamid 150 mg/m2 i. v. + dexametazon 24 mg i.v., v\u0161e 5 dn\u00ED po sob\u011B. Opakov\u00E1n\u00ED t\u011Bchto cykl\u016F bylo pl\u00E1nov\u00E1no v m\u011Bs\u00ED\u010Dn\u00EDch intervalech. Chemoterapii jsme podali 4kr\u00E1t ve v\u00FD\u0161e uveden\u00E9m slo\u017Een\u00ED a 3kr\u00E1t s aplikac\u00ED omezenou pouze na 1.-3. den. D\u016Fvodem pro redukci byla neutropenie grade III. Po ukon\u010Den\u00ED l\u00E9\u010Dby vymizely v\u0161echny symptomy nemoci, p\u0159etrv\u00E1v\u00E1 pouze diabetes insipidus, proto\u017Ee po\u0161kozen\u00ED stopky hypof\u00FDzy touto chorobou je ireverzibiln\u00ED. PET-CT prok\u00E1zalo dosa\u017Een\u00ED kompletn\u00ED remise nemoci a trv\u00E1n\u00ED t\u00E9to remise po 4,5 letech od zah\u00E1jen\u00ED l\u00E9\u010Dby. L\u00E9\u010Dba byla dob\u0159e tolerov\u00E1na, v pr\u016Fb\u011Bhu l\u00E9\u010Dby se nevyskytly \u017E\u00E1dn\u00E9 komplikace, vynucuj\u00EDc\u00ED si hospitalizaci. Po 4,5 letech od ukon\u010Den\u00ED l\u00E9\u010Dby p\u0159etrv\u00E1v\u00E1 m\u00EDrn\u00E1 trombocytopenie a neutropenie. Na z\u00E1klad\u011B popsan\u00E9ho p\u0159\u00EDpadu a citovan\u00E9 literatury pova\u017Eujeme kladribin za vhodn\u00FD l\u00E9k prvn\u00ED linie pro l\u00E9\u010Dbu Erdheimovy-Chesterovy choroby." . . "RIV/00209805:_____/14:#0000549!RIV15-MSM-00209805" . . "36331" . . "5-6" . "RIV/00209805:_____/14:#0000549" . "PET-CT dokumentovan\u00E1 kompletn\u00ED remise Erdheimovy-Chesterovy nemoci, trvaj\u00EDc\u00ED ji\u017E \u010Dty\u0159i roky od zah\u00E1jen\u00ED l\u00E9\u010Dby kladribinem" . . "0042-773X" . "Koukalov\u00E1, Renata" .