. . "Pekov\u00E1, So\u0148a" . "RIV/00023884:_____/08:#0001981" . . "0145-2126" . . "[3B8F8D899756]" . "Esenci\u00E1ln\u00ED trombocyt\u00E9mie (ET), chronick\u00E9 myeloproliferativn\u00ED onemocn\u011Bn\u00ED s absenc\u00ED Philadelphsk\u00E9ho chromosomu, je obykle onemocn\u011Bn\u00ED st\u0159edn\u00EDho v\u011Bku, a extr\u00E9mn\u011B z\u0159\u00EDdka se vyskytuje u pediatrick\u00FDch pacient\u016F. V t\u00E9to studii prezentujeme 12 d\u011Bt\u00ED s esenci\u00E1ln\u00ED trombocyt\u00E9mi\u00ED a jedno d\u00EDt\u011B s trombocytozou a rodinn\u00FDm v\u00FDskytem ET. Neprok\u00E1zali jsme mutaci JAK2 V617F v perifern\u00EDch leukocytech ani v separovan\u00FDch desti\u010Dk\u00E1ch a granulocytech. Monoklon\u00E1ln\u00ED hematopoeza byla prok\u00E1z\u00E1na pouze u jedn\u00E9 pacientky. Erytroidn\u00ED progenitory u v\u011Bt\u0161iny pacient\u016F vykazovaly hypersensitivitu k erytropoetinu (Epo) in vitro; Epo-nez\u00E1visl\u00E9 erytroidn\u00ED kolonie (EECS) byly zji\u0161t\u011Bny u 7 pacient\u016F. U t\u0159ech pacient\u016F jsme mezi EECS pozorovali kolonie heterozygotn\u00ED nebo homozygotn\u00ED v JAK2 V617F mutaci. Z uveden\u00FDch dat vypl\u00FDv\u00E1, \u017Ee u d\u011Btsk\u00FDch pacient\u016F s ET se vyskytuj\u00ED velmi vz\u00E1cn\u011B subklony nesouc\u00ED mutaci JAK2 V617F."@cs . "N" . . "Most pediatric patients with essential thrombocythemia show hypersensitivity to erythropoietin in vitro, with rare JAK2"@en . . "32" . "V\u011Bt\u0161ina d\u011Btsk\u00FDch pacient\u016F s esenci\u00E1ln\u00ED trombocyt\u00E9mi\u00ED vykazuje in vitro hypersensitivitu k erytropoetinu se vz\u00E1cn\u00FDmi JAK2"@cs . "GB - Spojen\u00E9 kr\u00E1lovstv\u00ED Velk\u00E9 Brit\u00E1nie a Severn\u00EDho Irska" . "RIV/00023884:_____/08:#0001981!RIV12-MZ0-00023884" . . "Essential thrombocythemia (ET), a Philadelphia (Ph) chromosome negative chronic myeloproliferative disorder, is usually a disease of middle age and it is extremely rare in pediatric patients. In this report we studied 12 children diagnosed with ET and one child with thrombocytosis and family history of ET. We failed to detect JAK2 V617F mutation either in peripheral blood leukocytes or in separated platelets and granulocytes. Monoclonal hematopoiesis was noted in only one female patient. Erythroid progenitors of most of the patients displayed hypersensitivity to erythropoietin (Epo) in vitro; Epo-independent erythroid colonies (EECs) were detected in seven patients. Among EECs of three patients we observed rare colonies heterozygous or homozygous for the JAK2 V617F mutation. Our data suggest that childhood ET patients could bear minor JAK2 V617F-positive subclones."@en . . . "V\u011Bt\u0161ina d\u011Btsk\u00FDch pacient\u016F s esenci\u00E1ln\u00ED trombocyt\u00E9mi\u00ED vykazuje in vitro hypersensitivitu k erytropoetinu se vz\u00E1cn\u00FDmi JAK2"@cs . . "1"^^ . "4"^^ . . "essential thrombocythemia, children age, clonality, erythroid colonies, hypersen"@en . "Esenci\u00E1ln\u00ED trombocyt\u00E9mie (ET), chronick\u00E9 myeloproliferativn\u00ED onemocn\u011Bn\u00ED s absenc\u00ED Philadelphsk\u00E9ho chromosomu, je obykle onemocn\u011Bn\u00ED st\u0159edn\u00EDho v\u011Bku, a extr\u00E9mn\u011B z\u0159\u00EDdka se vyskytuje u pediatrick\u00FDch pacient\u016F. V t\u00E9to studii prezentujeme 12 d\u011Bt\u00ED s esenci\u00E1ln\u00ED trombocyt\u00E9mi\u00ED a jedno d\u00EDt\u011B s trombocytozou a rodinn\u00FDm v\u00FDskytem ET. Neprok\u00E1zali jsme mutaci JAK2 V617F v perifern\u00EDch leukocytech ani v separovan\u00FDch desti\u010Dk\u00E1ch a granulocytech. Monoklon\u00E1ln\u00ED hematopoeza byla prok\u00E1z\u00E1na pouze u jedn\u00E9 pacientky. Erytroidn\u00ED progenitory u v\u011Bt\u0161iny pacient\u016F vykazovaly hypersensitivitu k erytropoetinu (Epo) in vitro; Epo-nez\u00E1visl\u00E9 erytroidn\u00ED kolonie (EECS) byly zji\u0161t\u011Bny u 7 pacient\u016F. U t\u0159ech pacient\u016F jsme mezi EECS pozorovali kolonie heterozygotn\u00ED nebo homozygotn\u00ED v JAK2 V617F mutaci. Z uveden\u00FDch dat vypl\u00FDv\u00E1, \u017Ee u d\u011Btsk\u00FDch pacient\u016F s ET se vyskytuj\u00ED velmi vz\u00E1cn\u011B subklony nesouc\u00ED mutaci JAK2 V617F." . . "Most pediatric patients with essential thrombocythemia show hypersensitivity to erythropoietin in vitro, with rare JAK2"@en . "V\u011Bt\u0161ina d\u011Btsk\u00FDch pacient\u016F s esenci\u00E1ln\u00ED trombocyt\u00E9mi\u00ED vykazuje in vitro hypersensitivitu k erytropoetinu se vz\u00E1cn\u00FDmi JAK2" . "V\u011Bt\u0161ina d\u011Btsk\u00FDch pacient\u016F s esenci\u00E1ln\u00ED trombocyt\u00E9mi\u00ED vykazuje in vitro hypersensitivitu k erytropoetinu se vz\u00E1cn\u00FDmi JAK2" . "9"^^ . . . . "Leukemia Research" . "3" . "402590" . .