. . "Hemofagocytuj\u00EDc\u00ED lymfohistiocyt\u00F3za"@cs . . . "RIV/00209775:_____/10:#0000133" . "56" . "hemophagocytic lymphohistiocytosis; immunodeficiency; lymphoproliferation; macrophage activation syndrome; immunosuppressive theraphy; stem-cell transplantation"@en . "Mejst\u0159\u00EDkov\u00E1, E." . "CZ - \u010Cesk\u00E1 republika" . . . "Hemofagocytuj\u00EDc\u00ED lymfohistiocyt\u00F3za (HLH) je heterogenn\u00ED skupina onemocn\u011Bn\u00ED vznikaj\u00EDc\u00EDch na podklad\u011B specifick\u00E9 vrozen\u00E9 nebo z\u00EDskan\u00E9 poruchy imunitn\u00EDho syst\u00E9mu. Podkladem pro vznik syndromu HLH, typick\u00FDch klinick\u00FDch a laboratorn\u00EDch p\u0159\u00EDznak\u016F, je aktivace a nekontrolovan\u00E1 proliferace T-lymfocyt\u016F a makrof\u00E1g\u016F prov\u00E1zen\u00E1 masivn\u00ED produkc\u00ED cytokin\u016F a hemofagocyt\u00E1rn\u00ED aktivitou makrof\u00E1g\u016F v lymforetikul\u00E1rn\u00EDm syst\u00E9mu a CNS. Na\u0161e zku\u0161enosti s diagnostikou a l\u00E9\u010Dbou HLH p\u0159edstavujeme na souboru 17 pacient\u016F s prim\u00E1rn\u00ED HLH, diagnostikovan\u00FDch a l\u00E9\u010Den\u00FDch v \u010CR v letech 1999-2009, jejich\u017E osud je srovnateln\u00FD s daty publikovan\u00FDmi evropskou HLH skupinou. Diagnostick\u00E1 dilemata dokladujeme podrobn\u011Bji na n\u011Bkolika kazuistik\u00E1ch prim\u00E1rn\u00ED i sekund\u00E1rn\u00ED HLH, demonstruj\u00EDc\u00EDch z\u00E1sadn\u00ED v\u00FDznam klinick\u00FDch zku\u0161enost\u00ED, znalosti diferenci\u00E1ln\u00ED diagn\u00F3zy a vyu\u017Eit\u00ED modern\u00EDch imunologick\u00FDch metod. Nejen na\u0161e zku\u0161enosti potvrzuj\u00ED, \u017Ee v\u010Dasn\u00E9 zah\u00E1jen\u00ED l\u00E9\u010Dby, tedy rychl\u00E1 diagnostika, je z\u00E1sadn\u00EDm prognostick\u00FDm faktorem u tohoto z\u00E1va\u017En\u00E9ho onemocn\u011Bn\u00ED." . . "Hemophagocytic lymphohistiocytosis syndrome"@en . "Hemofagocytuj\u00EDc\u00ED lymfohistiocyt\u00F3za (HLH) je heterogenn\u00ED skupina onemocn\u011Bn\u00ED vznikaj\u00EDc\u00EDch na podklad\u011B specifick\u00E9 vrozen\u00E9 nebo z\u00EDskan\u00E9 poruchy imunitn\u00EDho syst\u00E9mu. Podkladem pro vznik syndromu HLH, typick\u00FDch klinick\u00FDch a laboratorn\u00EDch p\u0159\u00EDznak\u016F, je aktivace a nekontrolovan\u00E1 proliferace T-lymfocyt\u016F a makrof\u00E1g\u016F prov\u00E1zen\u00E1 masivn\u00ED produkc\u00ED cytokin\u016F a hemofagocyt\u00E1rn\u00ED aktivitou makrof\u00E1g\u016F v lymforetikul\u00E1rn\u00EDm syst\u00E9mu a CNS. Na\u0161e zku\u0161enosti s diagnostikou a l\u00E9\u010Dbou HLH p\u0159edstavujeme na souboru 17 pacient\u016F s prim\u00E1rn\u00ED HLH, diagnostikovan\u00FDch a l\u00E9\u010Den\u00FDch v \u010CR v letech 1999-2009, jejich\u017E osud je srovnateln\u00FD s daty publikovan\u00FDmi evropskou HLH skupinou. Diagnostick\u00E1 dilemata dokladujeme podrobn\u011Bji na n\u011Bkolika kazuistik\u00E1ch prim\u00E1rn\u00ED i sekund\u00E1rn\u00ED HLH, demonstruj\u00EDc\u00EDch z\u00E1sadn\u00ED v\u00FDznam klinick\u00FDch zku\u0161enost\u00ED, znalosti diferenci\u00E1ln\u00ED diagn\u00F3zy a vyu\u017Eit\u00ED modern\u00EDch imunologick\u00FDch metod. Nejen na\u0161e zku\u0161enosti potvrzuj\u00ED, \u017Ee v\u010Dasn\u00E9 zah\u00E1jen\u00ED l\u00E9\u010Dby, tedy rychl\u00E1 diagnostika, je z\u00E1sadn\u00EDm prognostick\u00FDm faktorem u tohoto z\u00E1va\u017En\u00E9ho onemocn\u011Bn\u00ED."@cs . "Form\u00E1nkov\u00E1, R." . . "Hemophagocytic lymphohistiocytosis (HLH) represents a heterogenous group of specific immune-systeme deficiencies, characterized by uncontrolled proliferation of T lymphocytes and macrophages, resulting in overproduction of cytokines and inadequate hemophagocytic activity in lymphoreticular systeme and CNS. A series of 17 primary HLH patients diagnosed in the Czech Republic between 1999 and 2009, representing our experience with this rare disease, has outcome comparable to data published by European HLH group (4yrs OS 74%, EFS 56%). Diagnostic dilemma in HLH is demonstrated through several case reports of both primary and secondary HLH patients, revealing significance of clinical experience, awareness of differential diagnosis and application of modern immunological analyses in diagnostic procedure in HLH. All these parametres contribute to early onset of treatment determinating prognosis of HLH patients."@en . . "Hemofagocytuj\u00EDc\u00ED lymfohistiocyt\u00F3za" . "13"^^ . "Hemofagocytuj\u00EDc\u00ED lymfohistiocyt\u00F3za"@cs . . "9"^^ . "Sukov\u00E1, M." . "Sumerauer, D." . . . . "Vodi\u010Dkov\u00E1, E." . "Star\u00FD, J." . "RIV/00209775:_____/10:#0000133!RIV11-MZ0-00209775" . . "P(NS10480), S, V, Z(MSM0021620812), Z(MZ0FNM2005)" . "Sedl\u00E1\u010Dek, P." . "Hemofagocytuj\u00EDc\u00ED lymfohistiocyt\u00F3za" . "Freiberger, Tom\u00E1\u0161" . . "0042-773X" . . "1"^^ . . . . "261376" . "[87F53375DFB3]" . . . "Hemophagocytic lymphohistiocytosis syndrome"@en . . "\u0160p\u00ED\u0161ek, R." . . "Suppl 2" . "Vnit\u0159n\u00ED lk\u00E9ka\u0159stv\u00ED" .