. . . "Treatment of Langerhans Cells Histiocytosis by Cladribin Reached Long-Term Complete Remission in 9 out of 10 Adult Patients"@en . . . "The effectiveness of cladribine depends on the ratio of activating (deoxycytidine kinase) and inactivating (5-nucleotidase) enzymes. Not only is this ratio high in resting lymphocytes but also in Langerhans cells as well in some other histiocytic cells. Therefore, cladribine shows high effectiveness in patients with Langerhans cell histiocytosis (LCH). In 2003, the first report on excellent results with cladribine in first line treatment of patients with multisystem or multifocal LCH was published. That is why we use cladribine for adult patients with relapsing form of LCH and also for first line treatment of multifocal and multisystem LCH at our department. Patients and Methods: Since 2001, we have treated altogether 10 adults (9 male and 1 female) with cladribine. The median age at diagnosis was 31.5 years (range: 5\u201345). The multiorgan form of the disease was present in 8 patients, and 2 patients had the multifocal skeletal form with aggressive disease course."@en . "cladribine; 2-chlorodeoxyadenosine; Langerhans cell histiocytosis; diabetes insipidus"@en . "Kr\u00E1l, Zden\u011Bk" . . . "\u0158eh\u00E1k, Zden\u011Bk" . "RIV/00216224:14110/12:00060978!RIV13-MZ0-14110___" . . "RIV/00216224:14110/12:00060978" . . "4" . "25" . "L\u00E9\u010Dba histiocyt\u00F3zy z Langerhansov\u00FDch bun\u011Bk kladribinem dos\u00E1hla u 9 z 10 dosp\u011Bl\u00FDch pacient\u016F dlouhodob\u00E9 kompletn\u00ED remise"@cs . "\u00DA\u010Dinnost kladribinu z\u00E1vis\u00ED na pom\u011Bru aktiva\u010Dn\u00EDho enzymu (deoxycytidinkin\u00E1zy) a deaktiva\u010Dn\u00EDho enzymu (5-nukleotid\u00E1zy). Tento pom\u011Br je vysok\u00FD nejen v klidov\u00FDch lymfocytech, ale tak\u00E9 v Langerhansov\u00FDch bu\u0148k\u00E1ch a v n\u011Bkter\u00FDch dal\u0161\u00EDch histiocyt\u00E1rn\u00EDch bu\u0148k\u00E1ch. Proto m\u00E1 kladribin vysokou \u00FA\u010Dinnost u pacient\u016F s histiocyt\u00F3zou z Langerhansov\u00FDch bun\u011Bk (LCH). V roce 2003 byla zve\u0159ejn\u011Bna prvn\u00ED zpr\u00E1va o vynikaj\u00EDc\u00EDch v\u00FDsledc\u00EDch aplikace kladribinu v prvn\u00ED l\u00E9\u010Debn\u00E9 linii u pacient\u016F s multisyst\u00E9movou \u010Di multifok\u00E1ln\u00ED formou LCH. Proto na na\u0161em pracovi\u0161ti pou\u017E\u00EDv\u00E1me kladribin nejen pro dosp\u011Bl\u00E9 nemocn\u00E9 s relabuj\u00EDc\u00ED formou LCH, ale tak\u00E9 v r\u00E1mci l\u00E9\u010Dby prvn\u00ED linie multifok\u00E1ln\u00ED a multisyst\u00E9mov\u00E9 formy LCH. Soubor pacient\u016F a metody: Od roku 2001 jsme l\u00E9\u010Dili kladribinem celkem 10 dosp\u011Bl\u00FDch (9 mu\u017E\u016F a 1 \u017Eena). Medi\u00E1n v\u011Bku stanoven\u00ED diagn\u00F3zy je 31,5 (5\u201345) roku. Choroba m\u011Bla u 8 pacient\u016F formu multiorg\u00E1nov\u00E9ho posti\u017Een\u00ED a u 2 pacient\u016F formu multifok\u00E1ln\u00ED kostn\u00ED formy s agresivn\u00EDm pr\u016Fb\u011Bhem. Kladribin jsme pod\u00E1vali v d\u00E1vce 5 mg/m2/den s.c." . "L\u00E9\u010Dba histiocyt\u00F3zy z Langerhansov\u00FDch bun\u011Bk kladribinem dos\u00E1hla u 9 z 10 dosp\u011Bl\u00FDch pacient\u016F dlouhodob\u00E9 kompletn\u00ED remise" . . "Adam, Zden\u011Bk" . "Koukalov\u00E1, R." . "\u010Eura\u0161, J." . . "11"^^ . . "H\u00E1jek, Roman" . "L\u00E9\u010Dba histiocyt\u00F3zy z Langerhansov\u00FDch bun\u011Bk kladribinem dos\u00E1hla u 9 z 10 dosp\u011Bl\u00FDch pacient\u016F dlouhodob\u00E9 kompletn\u00ED remise"@cs . "P(LC06027), P(NS10408), P(NT11154), P(NT12130), P(NT12215), Z(MSM0021622434)" . "Treatment of Langerhans Cells Histiocytosis by Cladribin Reached Long-Term Complete Remission in 9 out of 10 Adult Patients"@en . "Krej\u010D\u00ED, Marta" . "14110" . "CZ - \u010Cesk\u00E1 republika" . . "Pour, Lud\u011Bk" . "Szturz, Petr" . "Navr\u00E1til, Milan" . . . "7"^^ . . "9"^^ . . . . . . . . . "Klinick\u00E1 onkologie" . "[DCE7A63D4BBA]" . . . "0862-495X" . . . "L\u00E9\u010Dba histiocyt\u00F3zy z Langerhansov\u00FDch bun\u011Bk kladribinem dos\u00E1hla u 9 z 10 dosp\u011Bl\u00FDch pacient\u016F dlouhodob\u00E9 kompletn\u00ED remise" . "Mayer, Ji\u0159\u00ED" . . "\u00DA\u010Dinnost kladribinu z\u00E1vis\u00ED na pom\u011Bru aktiva\u010Dn\u00EDho enzymu (deoxycytidinkin\u00E1zy) a deaktiva\u010Dn\u00EDho enzymu (5-nukleotid\u00E1zy). Tento pom\u011Br je vysok\u00FD nejen v klidov\u00FDch lymfocytech, ale tak\u00E9 v Langerhansov\u00FDch bu\u0148k\u00E1ch a v n\u011Bkter\u00FDch dal\u0161\u00EDch histiocyt\u00E1rn\u00EDch bu\u0148k\u00E1ch. Proto m\u00E1 kladribin vysokou \u00FA\u010Dinnost u pacient\u016F s histiocyt\u00F3zou z Langerhansov\u00FDch bun\u011Bk (LCH). V roce 2003 byla zve\u0159ejn\u011Bna prvn\u00ED zpr\u00E1va o vynikaj\u00EDc\u00EDch v\u00FDsledc\u00EDch aplikace kladribinu v prvn\u00ED l\u00E9\u010Debn\u00E9 linii u pacient\u016F s multisyst\u00E9movou \u010Di multifok\u00E1ln\u00ED formou LCH. Proto na na\u0161em pracovi\u0161ti pou\u017E\u00EDv\u00E1me kladribin nejen pro dosp\u011Bl\u00E9 nemocn\u00E9 s relabuj\u00EDc\u00ED formou LCH, ale tak\u00E9 v r\u00E1mci l\u00E9\u010Dby prvn\u00ED linie multifok\u00E1ln\u00ED a multisyst\u00E9mov\u00E9 formy LCH. Soubor pacient\u016F a metody: Od roku 2001 jsme l\u00E9\u010Dili kladribinem celkem 10 dosp\u011Bl\u00FDch (9 mu\u017E\u016F a 1 \u017Eena). Medi\u00E1n v\u011Bku stanoven\u00ED diagn\u00F3zy je 31,5 (5\u201345) roku. Choroba m\u011Bla u 8 pacient\u016F formu multiorg\u00E1nov\u00E9ho posti\u017Een\u00ED a u 2 pacient\u016F formu multifok\u00E1ln\u00ED kostn\u00ED formy s agresivn\u00EDm pr\u016Fb\u011Bhem. Kladribin jsme pod\u00E1vali v d\u00E1vce 5 mg/m2/den s.c."@cs . "146678" . . .