"Szturz, Petr" . "291715" . . . . . "V" . . "Systemic mastocytosis"@en . "6"^^ . "Koz\u00E1k, Tom\u00E1\u0161" . "Va\u0161k\u016F, Vladim\u00EDr" . "[33CFC8AD3E33]" . "3"^^ . . "Syst\u00E9mov\u00E1 mastocyt\u00F3za" . "RIV/00216224:14110/10:00046898!RIV11-MSM-14110___" . "Syst\u00E9mov\u00E1 mastocyt\u00F3za" . "Systemic mastocytosis is a heterogenous disorder characterized by abnormal growth and accumulation of abnormal mast cells in one or more organs. Mast cells are derived from CD34+/KIT+ pluripotent hematopoietic cells in the bone marrow. Most adult mastocytosis patients carry gain-of-function c-kit receptor mutations, most commonly D816V in the tyrosine kinase domain. The clinical phenotype of systemic mastocytosis is variable. Many of patients are suffering from urticaria pigmentosa, followed by the flushing, cramping, abdominal pain, diarrhea, bone pain and hepatosplenomegaly. Most adult patients have systemic disease (a condition generally confirmed by bone marrow biopsy). The natural history of systemic mastocytosis ranges from indolent forms to very aggressive subtypes."@en . . . "Syst\u00E9mov\u00E1 mastocyt\u00F3za"@cs . "7"^^ . . "K\u0159en, L." . "56" . "RIV/00216224:14110/10:00046898" . "HR - Chorvatsk\u00E1 republika" . . "0042-773X" . . "Syst\u00E9mov\u00E1 mastocyt\u00F3za"@cs . . "Syst\u00E9mov\u00E1 mastocyt\u00F3za je heterogenn\u00ED onemocn\u011Bn\u00ED charakterizovan\u00E9 mno\u017Een\u00EDm a akumulac\u00ED patologick\u00FDch mastocyt\u016F v jednom nebo v\u00EDce org\u00E1nech. Mastocyty vych\u00E1z\u00ED z CD34+/KIT+ pluripotentn\u00EDch hematopoetick\u00FDch bun\u011Bk z kostn\u00ED d\u0159en\u011B. Mnoho dosp\u011Bl\u00FDch nemocn\u00FDch s mastocyt\u00F3zou m\u00E1 v mastocytech aktiva\u010Dn\u00ED mutaci genu pro receptor c-kit, nej\u010Dast\u011Bji D816V v tyrozinkin\u00E1zov\u00E9 dom\u00E9n\u011B. Fenotyp syst\u00E9mov\u00E9 mastocyt\u00F3zy je r\u016Fznorod\u00FD. Mnoho nemocn\u00FDch m\u00E1 ko\u017En\u00ED zm\u011Bny typu urticaria pigmentosa, kter\u00E9 prov\u00E1z\u00ED \u010Derven\u00E1n\u00ED k\u016F\u017Ee, k\u0159e\u010De, bolesti b\u0159icha, pr\u016Fjem, bolesti kost\u00ED a hepatosplenomegalie. Zejm\u00E9na dosp\u011Bl\u00ED nemocn\u00ED maj\u00ED velmi \u010Dasto syst\u00E9mov\u00E9 posti\u017Een\u00ED, kter\u00E9 je povrzeno biopsi\u00ED kostn\u00ED d\u0159en\u011B. Pr\u016Fb\u011Bh nemoci je u n\u011Bkter\u00FDch typ\u016F indolentn\u00ED, u jin\u00FDch m\u016F\u017Ee b\u00FDt velmi agresivn\u00ED." . . . "Suppl 2" . "Doubek, Michael" . "Systemic mastocytosis"@en . . . "diagnostics; KIT; management; masitinib; systemic mastocytosis"@en . "14110" . . "Syst\u00E9mov\u00E1 mastocyt\u00F3za je heterogenn\u00ED onemocn\u011Bn\u00ED charakterizovan\u00E9 mno\u017Een\u00EDm a akumulac\u00ED patologick\u00FDch mastocyt\u016F v jednom nebo v\u00EDce org\u00E1nech. Mastocyty vych\u00E1z\u00ED z CD34+/KIT+ pluripotentn\u00EDch hematopoetick\u00FDch bun\u011Bk z kostn\u00ED d\u0159en\u011B. Mnoho dosp\u011Bl\u00FDch nemocn\u00FDch s mastocyt\u00F3zou m\u00E1 v mastocytech aktiva\u010Dn\u00ED mutaci genu pro receptor c-kit, nej\u010Dast\u011Bji D816V v tyrozinkin\u00E1zov\u00E9 dom\u00E9n\u011B. Fenotyp syst\u00E9mov\u00E9 mastocyt\u00F3zy je r\u016Fznorod\u00FD. Mnoho nemocn\u00FDch m\u00E1 ko\u017En\u00ED zm\u011Bny typu urticaria pigmentosa, kter\u00E9 prov\u00E1z\u00ED \u010Derven\u00E1n\u00ED k\u016F\u017Ee, k\u0159e\u010De, bolesti b\u0159icha, pr\u016Fjem, bolesti kost\u00ED a hepatosplenomegalie. Zejm\u00E9na dosp\u011Bl\u00ED nemocn\u00ED maj\u00ED velmi \u010Dasto syst\u00E9mov\u00E9 posti\u017Een\u00ED, kter\u00E9 je povrzeno biopsi\u00ED kostn\u00ED d\u0159en\u011B. Pr\u016Fb\u011Bh nemoci je u n\u011Bkter\u00FDch typ\u016F indolentn\u00ED, u jin\u00FDch m\u016F\u017Ee b\u00FDt velmi agresivn\u00ED."@cs . "Tich\u00E1, Marie" . "Vnit\u0159n\u00ED l\u00E9ka\u0159stv\u00ED" . .