"\u00DAvod: Erdheimova-Chesterova nemoc je velmi vz\u00E1cn\u00E1 choroba dosp\u011Bl\u00FDch. Typicky zp\u016Fsobuje hyperost\u00F3zu dlouh\u00FDch kon\u010Detin, fibr\u00F3zu retroperitonea, zes\u00EDlen\u00ED st\u011Bny aorty a m\u016F\u017Ee tvo\u0159it i infiltr\u00E1ty ve stopce hypof\u00FDzy a v dal\u0161\u00EDch \u010D\u00E1stech mozku. Pacienti \u010Dasto m\u00EDvaj\u00ED teploty zp\u016Fsoben\u00E9 touto nemoc\u00ED a bolesti v doln\u00EDch kon\u010Detin\u00E1ch. Vz\u00E1cnost t\u011Bchto p\u0159\u00EDpad\u016F je d\u016Fvodem, pro\u010D pro l\u00E9\u010Dbu t\u00E9to nemoci neexistuj\u00ED \u017E\u00E1dn\u00E1 doporu\u010Den\u00ED. Proto popisujeme na\u0161e zku\u0161enosti s aplikac\u00ED lenalidomidu u pacienta s malou l\u00E9\u010Debnou odpov\u011Bd\u00ED po 2-chlorodeoxyadenosinu. P\u0159\u00EDpad: Prvn\u00EDm p\u0159\u00EDznakem nemoci byl diabetes insipidus a v pr\u016Fb\u011Bhu 4 let postupn\u011B se rozv\u00EDjej\u00EDc\u00ED neurologick\u00E9 probl\u00E9my (dysartrie, pravostrann\u00E1 hemipar\u00E9za). Diagn\u00F3za byla stanovena histologick\u00FDm vy\u0161et\u0159en\u00EDm kostn\u00ED d\u0159en\u011B lopaty kosti p\u00E1nevn\u00ED. V dob\u011B stanoven\u00ED diagn\u00F3zy m\u011Bl nemocn\u00FD ji\u017E v\u00EDce\u010Detn\u00E9 infiltr\u00E1ty v mozku, zes\u00EDlenou st\u011Bnu hrudn\u00ED a b\u0159i\u0161n\u00ED aorty, fibrotick\u00E9 zm\u011Bny v retroperitoneu a typickou hyperost\u00F3zu dlouh\u00FDch kost\u00ED doln\u00EDch kon\u010Detin s vysokou akumulac\u00ED jak techneciumpyrofosf\u00E1tu, tak i fluorodeoxygluk\u00F3zy. L\u00E9\u010Dbu jsme zah\u00E1jili monoterapi\u00ED 2-chlorodeoxyadenosinem 5 mg/m2 s.c. 5 dn\u00ED po sob\u011B ve 28denn\u00EDch cyklech. V pr\u016Fb\u011Bhu t\u0159et\u00EDho cyklu na proveden\u00E9m MRI mozku nebyla z\u0159eteln\u00E1 l\u00E9\u010Debn\u00E1 odpov\u011B\u010F, a tak 4.\u20136. cyklus prob\u00EDhal ve slo\u017Een\u00ED 2-chlorodeoxyadenosin 5 mg/m2 + cyklofosfamid 150 mg/m2 + dexametazon 24 mg 1.\u20135. den ve 28denn\u00EDm cyklu. Po 6 cyklech bylo provedeno kontroln\u00ED MRI vy\u0161et\u0159en\u00ED mozku, kter\u00E9 prok\u00E1zalo parci\u00E1ln\u00ED regresi lo\u017Eisek. Kontroln\u00ED PET-CT vy\u0161et\u0159en\u00ED v\u0161ak prok\u00E1zalo n\u00E1r\u016Fst akumulace fluorodeoxygluk\u00F3zy v kostn\u00EDch lo\u017Eisc\u00EDch nemoci. V r\u00E1mci l\u00E9\u010Dby druh\u00E9 linie byl pod\u00E1n lenalidomid 25 mg denn\u011B 1.\u201321. den ve 28denn\u00EDch cyklech. Tolerance lenalidomidu byla excelentn\u00ED, po\u010Det neutrofil\u016F a trombocyt\u016F byl po celou dobu l\u00E9\u010Dby ve fyziologick\u00E9m rozmez\u00ED. Kontroln\u00ED MRI vy\u0161et\u0159en\u00ED prok\u00E1zalo \u00FApln\u00E9 vymizen\u00ED lo\u017Eisek v mozku. Kontroln\u00ED PET-CT vy\u0161et\u0159en\u00ED v\u0161ak prok\u00E1zalo dal\u0161\u00ED n\u00E1r\u016Fst akumulace fluorodeoxygluk\u00F3zy v kostn\u00EDch lo\u017Eisc\u00EDch doln\u00EDch kon\u010Detin."@cs . "Parci\u00E1ln\u00ED regrese lo\u017Eisek Erdheimovy-Chesterovy nemoci v CNS po l\u00E9\u010Db\u011B 2-chlorodeoxyadenosinem a jejich kompletn\u00ED vymizen\u00ED p\u0159i l\u00E9\u010Db\u011B lenalidomidem"@cs . . . . . . . "Parci\u00E1ln\u00ED regrese lo\u017Eisek Erdheimovy-Chesterovy nemoci v CNS po l\u00E9\u010Db\u011B 2-chlorodeoxyadenosinem a jejich kompletn\u00ED vymizen\u00ED p\u0159i l\u00E9\u010Db\u011B lenalidomidem" . "Klinick\u00E1 onkologie" . "17"^^ . . . . "Parci\u00E1ln\u00ED regrese lo\u017Eisek Erdheimovy-Chesterovy nemoci v CNS po l\u00E9\u010Db\u011B 2-chlorodeoxyadenosinem a jejich kompletn\u00ED vymizen\u00ED p\u0159i l\u00E9\u010Db\u011B lenalidomidem"@cs . . . . . "Adam, Zden\u011Bk" . . . . "Koukalov\u00E1, Renata" . "0862-495X" . "Partial regression of CNS lesions of Erdheim-Chester Disease after treatment with 2-chlorodeoxadenosine and their full remission following treatment with lenalidomide"@en . "219585" . . . "Erdheim-Chester disease; juvenile xanthogranuloma; osteosclerosis; skeletal scinigraphy; PET-CT; lenalidomide; 2-chlorodeoxyadenosine; cladribin; retroperitoneal fibrosis"@en . . "Partial regression of CNS lesions of Erdheim-Chester Disease after treatment with 2-chlorodeoxadenosine and their full remission following treatment with lenalidomide"@en . "Introduction: Erdheim-Chester disease is a very rare syndrome affecting adult population. It typically causes hyperostosis of long bones, retroperitoneal fibrosis and widening of the aortic wall. Patients frequently suffer from disease-associated fevers and pain in the lower limbs. No guidelines are available for the treatment of this rare ailment. Therefore, we describe our experience with lenalidomide in a patient with poor treatment response to 2-chlorodeoxyadenosine. Case: Diabetes insipidus and neurological problems developing over 4 years were the first signs of the disease. The disease was diagnosed from histology of the bone marrow extracted from the ilium. At diagnosis, the patient had multiple infiltrates in the brain, widened wall of the thoracic and abdominal aorta, fibrotic changes to retroperitoneum and typical hyperostosis of the long bones of lower limbs with high accumulation of technetium pyrophosphate as well as fluorodeoxyglucose. First line treatment involved 2-chlorodeoxyadenosine 5 mg/m2 s.c. for 5 consecutive days every 28 days. There was no clear treatment response identifiable on the MR scan of the brain following the third cycle and thus 4th\u20136th cycle consisted of 2-chlorodexyadenosine 5 mg/m2 + cyclophosphamide 150 mg/m2 + dexamethasone 24 mg day 1\u20135 every 28 days. After the 6th cycle, MR showed partial regression of the brain lesions. PET-CT showed an increased accumulation of fluorodeoxyglucose in bone lesions. Second line treatment involved lenalidomide 25 mg/day days 1\u201321 every 28 days. Lenalidomide tolerance was excellent; the number of neutrophils and thrombocytes was within the physiological range throughout the treatment period. Follow-up MR showed complete remission of the brain lesions, while follow-up PET-CT showed further increase in fluorodeoxyglucose accumulation in the bones of lower limbs."@en . "http://www.linkos.cz/files/klinicka-onkologie/166/3879.pdf" . . . . "2"^^ . "524" . . "\u0158eh\u00E1k, Zden\u011Bk" . "RIV/00209805:_____/11:#0000201!RIV12-MZ0-00209805" . "\u00DAvod: Erdheimova-Chesterova nemoc je velmi vz\u00E1cn\u00E1 choroba dosp\u011Bl\u00FDch. Typicky zp\u016Fsobuje hyperost\u00F3zu dlouh\u00FDch kon\u010Detin, fibr\u00F3zu retroperitonea, zes\u00EDlen\u00ED st\u011Bny aorty a m\u016F\u017Ee tvo\u0159it i infiltr\u00E1ty ve stopce hypof\u00FDzy a v dal\u0161\u00EDch \u010D\u00E1stech mozku. Pacienti \u010Dasto m\u00EDvaj\u00ED teploty zp\u016Fsoben\u00E9 touto nemoc\u00ED a bolesti v doln\u00EDch kon\u010Detin\u00E1ch. Vz\u00E1cnost t\u011Bchto p\u0159\u00EDpad\u016F je d\u016Fvodem, pro\u010D pro l\u00E9\u010Dbu t\u00E9to nemoci neexistuj\u00ED \u017E\u00E1dn\u00E1 doporu\u010Den\u00ED. Proto popisujeme na\u0161e zku\u0161enosti s aplikac\u00ED lenalidomidu u pacienta s malou l\u00E9\u010Debnou odpov\u011Bd\u00ED po 2-chlorodeoxyadenosinu. P\u0159\u00EDpad: Prvn\u00EDm p\u0159\u00EDznakem nemoci byl diabetes insipidus a v pr\u016Fb\u011Bhu 4 let postupn\u011B se rozv\u00EDjej\u00EDc\u00ED neurologick\u00E9 probl\u00E9my (dysartrie, pravostrann\u00E1 hemipar\u00E9za). Diagn\u00F3za byla stanovena histologick\u00FDm vy\u0161et\u0159en\u00EDm kostn\u00ED d\u0159en\u011B lopaty kosti p\u00E1nevn\u00ED. V dob\u011B stanoven\u00ED diagn\u00F3zy m\u011Bl nemocn\u00FD ji\u017E v\u00EDce\u010Detn\u00E9 infiltr\u00E1ty v mozku, zes\u00EDlenou st\u011Bnu hrudn\u00ED a b\u0159i\u0161n\u00ED aorty, fibrotick\u00E9 zm\u011Bny v retroperitoneu a typickou hyperost\u00F3zu dlouh\u00FDch kost\u00ED doln\u00EDch kon\u010Detin s vysokou akumulac\u00ED jak techneciumpyrofosf\u00E1tu, tak i fluorodeoxygluk\u00F3zy. L\u00E9\u010Dbu jsme zah\u00E1jili monoterapi\u00ED 2-chlorodeoxyadenosinem 5 mg/m2 s.c. 5 dn\u00ED po sob\u011B ve 28denn\u00EDch cyklech. V pr\u016Fb\u011Bhu t\u0159et\u00EDho cyklu na proveden\u00E9m MRI mozku nebyla z\u0159eteln\u00E1 l\u00E9\u010Debn\u00E1 odpov\u011B\u010F, a tak 4.\u20136. cyklus prob\u00EDhal ve slo\u017Een\u00ED 2-chlorodeoxyadenosin 5 mg/m2 + cyklofosfamid 150 mg/m2 + dexametazon 24 mg 1.\u20135. den ve 28denn\u00EDm cyklu. Po 6 cyklech bylo provedeno kontroln\u00ED MRI vy\u0161et\u0159en\u00ED mozku, kter\u00E9 prok\u00E1zalo parci\u00E1ln\u00ED regresi lo\u017Eisek. Kontroln\u00ED PET-CT vy\u0161et\u0159en\u00ED v\u0161ak prok\u00E1zalo n\u00E1r\u016Fst akumulace fluorodeoxygluk\u00F3zy v kostn\u00EDch lo\u017Eisc\u00EDch nemoci. V r\u00E1mci l\u00E9\u010Dby druh\u00E9 linie byl pod\u00E1n lenalidomid 25 mg denn\u011B 1.\u201321. den ve 28denn\u00EDch cyklech. Tolerance lenalidomidu byla excelentn\u00ED, po\u010Det neutrofil\u016F a trombocyt\u016F byl po celou dobu l\u00E9\u010Dby ve fyziologick\u00E9m rozmez\u00ED. Kontroln\u00ED MRI vy\u0161et\u0159en\u00ED prok\u00E1zalo \u00FApln\u00E9 vymizen\u00ED lo\u017Eisek v mozku. Kontroln\u00ED PET-CT vy\u0161et\u0159en\u00ED v\u0161ak prok\u00E1zalo dal\u0161\u00ED n\u00E1r\u016Fst akumulace fluorodeoxygluk\u00F3zy v kostn\u00EDch lo\u017Eisc\u00EDch doln\u00EDch kon\u010Detin." . "Parci\u00E1ln\u00ED regrese lo\u017Eisek Erdheimovy-Chesterovy nemoci v CNS po l\u00E9\u010Db\u011B 2-chlorodeoxyadenosinem a jejich kompletn\u00ED vymizen\u00ED p\u0159i l\u00E9\u010Db\u011B lenalidomidem" . . . "5" . . . "[9D9AB2CCAA85]" . "RIV/00209805:_____/11:#0000201" . . "N, P(NS10408), P(NT11154), P(NT12130), P(NT12215), Z(MZ0MOU2005)" . . "15"^^ . "CZ - \u010Cesk\u00E1 republika" .