"Diagnostika a l\u00E9\u010Dba BCR/ABL-negativn\u00EDch myeloproliferativn\u00EDch onemocn\u011Bn\u00ED - principy a v\u00FDchodiska doporu\u010Den\u00ED CZEMP" . "Campr, V." . . . . . . "Diagnostika a l\u00E9\u010Dba BCR/ABL-negativn\u00EDch myeloproliferativn\u00EDch onemocn\u011Bn\u00ED - principy a v\u00FDchodiska doporu\u010Den\u00ED CZEMP" . "V roce 2009 byla aktualizov\u00E1na a roz\u0161\u00ED\u0159ena doporu\u010Den\u00ED \u010Cesk\u00E9 pracovn\u00ED skupiny pro Ph negativn\u00ED (Ph-) myeloproliferativn\u00ED onemocn\u011Bn\u00ED (CZEMP) pro diagnostiku a l\u00E9\u010Dbu BCR/ABL-negativn\u00EDch myeloproliferativn\u00EDch onemocn\u011Bn\u00ED (MPO), tj. esenci\u00E1ln\u00ED trombocytemie (ET), polycythaemia vera (PV) a prim\u00E1rn\u00ED myelofibr\u00F3zy (PMF). V tomto \u010Dl\u00E1nku jsou podrobn\u011B rozvedena v\u00FDchodiska doporu\u010Den\u00FDch postup\u016F. Krit\u00E9ria CZEMP pro diagnostiku ET a PMF se op\u00EDraj\u00ED o histopatologick\u00FD (HP) n\u00E1lez, kter\u00FD v\u0161ak mus\u00ED b\u00FDt bezpodm\u00EDne\u010Dn\u011B v souladu s popsan\u00FDmi klinick\u00FDmi a laboratorn\u00EDmi charakteristikami u ET, resp. r\u016Fzn\u00FDch stadi\u00ED PMF. Po\u010Det trombocyt\u016F nen\u00ED pro diagn\u00F3zu rozhoduj\u00EDc\u00ED. U p\u0159\u00EDpad\u016F, u kter\u00FDch nen\u00ED k dispozici adekv\u00E1tn\u011B odebran\u00FD a ode\u010Dten\u00FD HP n\u00E1lez, doporu\u010Dujeme u\u017E\u00EDt krit\u00E9ri\u00ED Polycythemia Vera Study Group (PVSG). Diagnostika typick\u00E9 PV se op\u00EDr\u00E1 o pr\u016Fkaz mutace V617F genu JAK2 za p\u0159edpokladu v\u00FDznamn\u00E9ho zv\u00FD\u0161en\u00ED hodnot \u010Derven\u00E9ho krevn\u00EDho obrazu. P\u0159i jeho hrani\u010Dn\u00EDch hodnot\u00E1ch je nutn\u00FD pr\u016Fkaz zv\u00FD\u0161en\u00E9 celkov\u00E9 masy erytrocyt\u016F (RCM). U atypick\u00FDch p\u0159\u00EDpad\u016F, nen\u00ED-li p\u0159\u00EDtomna polyglobulie (anebo zv\u00FD\u0161en\u00E1 hodnota RCM), posta\u010Duje HP obraz PV dle definice WHO plus n\u00E1lez mutace V617F JAK2, anebo nen\u00ED-li p\u0159\u00EDtomna mutace JAK2 V617F, sta\u010D\u00ED HP obraz PV + pr\u016Fkaz polyglobulie (anebo zv\u00FD\u0161en\u00E9ho RCM). Principy l\u00E9\u010Dby ET i ostatn\u00EDch MPO s trombocytemi\u00ED (MPO-T; tj. ran\u00FDch stadi\u00ED PMF a PV) jsou identick\u00E9. Pacienti jsou stratifikov\u00E1ni podle p\u0159\u00EDtomnosti trombotick\u00E9ho rizika (p\u0159edchoz\u00ED tromb\u00F3zy, dal\u0161\u00EDho trombofiln\u00EDho stavu, mutace JAK2), p\u0159\u00EDtomnosti symptom\u016F onemocn\u011Bn\u00ED (obvykle mikrocirkula\u010Dn\u00EDch), po\u010Dtu trombocyt\u016F a v\u011Bku. Pouze pacienti do 65 let bez uveden\u00FDch rizik s po\u010Dtem trombocyt\u016F pod 1 000 109/\u2009l jsou pova\u017Eov\u00E1ni za n\u00EDzkorizikov\u00E9 a nevy\u017Eaduj\u00ED cytoreduktivn\u00ED l\u00E9\u010Dbu. Ostatn\u00ED jsou vysokorizikov\u00ED a jsou indikov\u00E1ni k tromboredukci. Od v\u011Bku nad 65 je mo\u017Eno u\u017E\u00EDt potenci\u00E1ln\u011B leukemogenn\u00ED hydroxyureu (HU), u mlad\u0161\u00EDch vol\u00EDme mezi anagrelidem (ANG) a interferonem-\u03B1 (IFN). U vysokorizikov\u00FDch nemocn\u00FDch je c\u00EDlem udr\u017Eet trombocyty trvale ..." . "194251" . "Diagnostika a l\u00E9\u010Dba BCR/ABL-negativn\u00EDch myeloproliferativn\u00EDch onemocn\u011Bn\u00ED - principy a v\u00FDchodiska doporu\u010Den\u00ED CZEMP"@cs . "57" . "16"^^ . . . "I, O, V" . "Posp\u00ED\u0161ilov\u00E1, D." . . "\u010Cern\u00E1, O." . "Nov\u00E1kov\u00E1, L." . "[065A274AE8BD]" . . "1"^^ . "Penka, M." . "0042-773X" . . "2" . . . "K\u0159en, L." . "CZ - \u010Cesk\u00E1 republika" . "Ko\u0159\u00EDstek, Z." . "RIV/00843989:_____/11:00102124" . "Jon\u00E1\u0161ov\u00E1, A." . "V roce 2009 byla aktualizov\u00E1na a roz\u0161\u00ED\u0159ena doporu\u010Den\u00ED \u010Cesk\u00E9 pracovn\u00ED skupiny pro Ph negativn\u00ED (Ph-) myeloproliferativn\u00ED onemocn\u011Bn\u00ED (CZEMP) pro diagnostiku a l\u00E9\u010Dbu BCR/ABL-negativn\u00EDch myeloproliferativn\u00EDch onemocn\u011Bn\u00ED (MPO), tj. esenci\u00E1ln\u00ED trombocytemie (ET), polycythaemia vera (PV) a prim\u00E1rn\u00ED myelofibr\u00F3zy (PMF). V tomto \u010Dl\u00E1nku jsou podrobn\u011B rozvedena v\u00FDchodiska doporu\u010Den\u00FDch postup\u016F. Krit\u00E9ria CZEMP pro diagnostiku ET a PMF se op\u00EDraj\u00ED o histopatologick\u00FD (HP) n\u00E1lez, kter\u00FD v\u0161ak mus\u00ED b\u00FDt bezpodm\u00EDne\u010Dn\u011B v souladu s popsan\u00FDmi klinick\u00FDmi a laboratorn\u00EDmi charakteristikami u ET, resp. r\u016Fzn\u00FDch stadi\u00ED PMF. Po\u010Det trombocyt\u016F nen\u00ED pro diagn\u00F3zu rozhoduj\u00EDc\u00ED. U p\u0159\u00EDpad\u016F, u kter\u00FDch nen\u00ED k dispozici adekv\u00E1tn\u011B odebran\u00FD a ode\u010Dten\u00FD HP n\u00E1lez, doporu\u010Dujeme u\u017E\u00EDt krit\u00E9ri\u00ED Polycythemia Vera Study Group (PVSG). Diagnostika typick\u00E9 PV se op\u00EDr\u00E1 o pr\u016Fkaz mutace V617F genu JAK2 za p\u0159edpokladu v\u00FDznamn\u00E9ho zv\u00FD\u0161en\u00ED hodnot \u010Derven\u00E9ho krevn\u00EDho obrazu. P\u0159i jeho hrani\u010Dn\u00EDch hodnot\u00E1ch je nutn\u00FD pr\u016Fkaz zv\u00FD\u0161en\u00E9 celkov\u00E9 masy erytrocyt\u016F (RCM). U atypick\u00FDch p\u0159\u00EDpad\u016F, nen\u00ED-li p\u0159\u00EDtomna polyglobulie (anebo zv\u00FD\u0161en\u00E1 hodnota RCM), posta\u010Duje HP obraz PV dle definice WHO plus n\u00E1lez mutace V617F JAK2, anebo nen\u00ED-li p\u0159\u00EDtomna mutace JAK2 V617F, sta\u010D\u00ED HP obraz PV + pr\u016Fkaz polyglobulie (anebo zv\u00FD\u0161en\u00E9ho RCM). Principy l\u00E9\u010Dby ET i ostatn\u00EDch MPO s trombocytemi\u00ED (MPO-T; tj. ran\u00FDch stadi\u00ED PMF a PV) jsou identick\u00E9. Pacienti jsou stratifikov\u00E1ni podle p\u0159\u00EDtomnosti trombotick\u00E9ho rizika (p\u0159edchoz\u00ED tromb\u00F3zy, dal\u0161\u00EDho trombofiln\u00EDho stavu, mutace JAK2), p\u0159\u00EDtomnosti symptom\u016F onemocn\u011Bn\u00ED (obvykle mikrocirkula\u010Dn\u00EDch), po\u010Dtu trombocyt\u016F a v\u011Bku. Pouze pacienti do 65 let bez uveden\u00FDch rizik s po\u010Dtem trombocyt\u016F pod 1 000 109/\u2009l jsou pova\u017Eov\u00E1ni za n\u00EDzkorizikov\u00E9 a nevy\u017Eaduj\u00ED cytoreduktivn\u00ED l\u00E9\u010Dbu. Ostatn\u00ED jsou vysokorizikov\u00ED a jsou indikov\u00E1ni k tromboredukci. Od v\u011Bku nad 65 je mo\u017Eno u\u017E\u00EDt potenci\u00E1ln\u011B leukemogenn\u00ED hydroxyureu (HU), u mlad\u0161\u00EDch vol\u00EDme mezi anagrelidem (ANG) a interferonem-\u03B1 (IFN). U vysokorizikov\u00FDch nemocn\u00FDch je c\u00EDlem udr\u017Eet trombocyty trvale ..."@cs . "Diagnosis and treatment of BCR/ABL-negative myeloproliferative diseases \u2013 principles and rationale of CZEMP recommendations"@en . . "Walterov\u00E1, L." . . "25"^^ . . "Ph-myeloproliferative disease; essential thrombocythemia; polycythaemia vera; primary myelofibrosis; diagnosis; risk factors; treatment algorithm; hydroxyurea; anagrelide; interferon-\u03B1; acetylsalicylic acid; hematopoetic steam cell transplantation"@en . "RIV/00843989:_____/11:00102124!RIV12-MZ0-00843989" . "Vonke, I." . . "Schwarz, J." . "Schutzov\u00E1, M." . "Diagnostika a l\u00E9\u010Dba BCR/ABL-negativn\u00EDch myeloproliferativn\u00EDch onemocn\u011Bn\u00ED - principy a v\u00FDchodiska doporu\u010Den\u00ED CZEMP"@cs . . . . . . . "Dul\u00ED\u010Dek, P." . "Vnit\u0159n\u00ED l\u00E9ka\u0159stv\u00ED" . "In 2009, the recommendations of the Czech Collaborative Group for Ph- Myeloproliferative Diseases (CZEMP) for diagnosis and treatment of BCR/ABL-negative myeloproliferative diseases (MPD), i.e. essential thrombocythemia (ET), polycythaemia vera (PV) and primary myelofibrosis (PMF) were updated and extended. The present article gives the rationale of the recommendations in full detail. The CZEMP diagnostic criteria for ET and PMF are based on histopathological (HP) findings, which must unconditionally be in line with the given clinical and laboratory characteristics of ET or of a certain stage of PMF, respectively. The platelet count is not decisive for diagnosis. In cases lacking an adequately taken and read HP finding, the Polycythemia Vera Study Group (PVSG) criteria are recommended. The diagnosis of typical PV is based on demonstration of the V617F mutation of the JAK2 gene along with a significant increase of red cell parameters. If these are close to borderline, the demonstration of increased total red cell mass (RCM) is required. In atypical cases lacking polyglobulia or elevated RCM, the HP picture of PV (in accordance with WHO description) plus JAK2 V617F mutation is satisfactory for diagnosis, or, in cases lacking JAK2 V617F mutation, the HP picture of PV along with polyglobulia (or increased RCM) is sufficient. The treatment principles of ET and other MPDs with thrombocythemia (MPD-T; i.e. the early stages of PMF and PV) are identical. The patients are stratified by their thrombotic risk (preceding thrombosis, another thrombophilic state, JAK2 mutation), presence of disease symptoms (mainly microcirculatory), platelet count and age. Only patients up to 65 years lacking the above mentioned risks with a platelet count < 1 000 109/\u2009l are considered as low-risk and do not demand cytoreducing therapy. The others are high-risk ones and have an indication for thromboreduction. In patients older than 65 years, the potentially leukemogenic drug hydroxyurea (HU)..."@en . . . "Brychtov\u00E1, Y." . . "Kissov\u00E1, J." . . "Bodz\u00E1sov\u00E1, Cec\u00EDlia" . "Diagnosis and treatment of BCR/ABL-negative myeloproliferative diseases \u2013 principles and rationale of CZEMP recommendations"@en .