Características clínicas y mortalidad de pacientes adultos con síndrome hemafagocítico, estudio de cohorte retrospectiva

Background: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation Aim: To describe the clinical characteristics, causes and survival associated with HLH. Material and Methods: Review of medical records of patients with HLH attended bet...

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Autores principales:	Warley,Fernando, Bonella,Belén M, Odstrcil-Bobillo,M. Silvina, Otero,Victoria, Waisman,Gabriel, Bendelman,Gisela, Giunta,Diego, Peuchot,Verónica, Ungaro,Catalina M
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2017
Materias:	Hemaphagocytic Immunosuppression Immunocompromised Host Lymphohistiocytosis
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872017000300008
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spelling	oai:scielo:S0034-988720170003000082017-05-18Características clínicas y mortalidad de pacientes adultos con síndrome hemafagocítico, estudio de cohorte retrospectivaWarley,FernandoBonella,Belén MOdstrcil-Bobillo,M. SilvinaOtero,VictoriaWaisman,GabrielBendelman,GiselaGiunta,DiegoPeuchot,VerónicaUngaro,Catalina M Hemaphagocytic Immunosuppression Immunocompromised Host Lymphohistiocytosis Background: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation Aim: To describe the clinical characteristics, causes and survival associated with HLH. Material and Methods: Review of medical records of patients with HLH attended between 2004 and 2016. They were classified according to their probable cause in: associated with immunosuppression, cancer, post-infectious or idiopathic. Kaplan-Meier survival analysis was performed. Results: Twenty seven patients with HLH aged 18 to 87 years (59% men), were detected. Fourteen (52%) were secondary to immunosuppression, six (22%) were post-infectious, five (18%) were associated with cancer and two (7%) were of unknown cause. There were no significant differences in clinical or laboratory features between these etiologies. Within the immunosuppressed group, 12 (86%) were patients with oncologic or hematologic diseases or bone marrow transplantation. Associated cancers were mostly oncohematologic diseases. Thirty-day mortality was 53.4% (95% confidence intervals (CI) 32.7-70.3%), despite the treatment. Mortality was significantly associated with the presence of renal failure with a hazard ratio (HR) of 3.4 (95% CI of 1.2-9.9, p =0.025). Treatment of the underlying disease proved to be protective against mortality with an HR of 0.3 (95% CI 0.1 to 0.98, p = 0.046). Conclusions: The prognosis of HLH could be related to the treatment of the underlying disease. The study of the pathophysiology of this syndrome will allow a better understanding and treatment.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.145 n.3 20172017-03-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872017000300008es10.4067/S0034-98872017000300008
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Hemaphagocytic Immunosuppression Immunocompromised Host Lymphohistiocytosis
spellingShingle	Hemaphagocytic Immunosuppression Immunocompromised Host Lymphohistiocytosis Warley,Fernando Bonella,Belén M Odstrcil-Bobillo,M. Silvina Otero,Victoria Waisman,Gabriel Bendelman,Gisela Giunta,Diego Peuchot,Verónica Ungaro,Catalina M Características clínicas y mortalidad de pacientes adultos con síndrome hemafagocítico, estudio de cohorte retrospectiva
description	Background: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation Aim: To describe the clinical characteristics, causes and survival associated with HLH. Material and Methods: Review of medical records of patients with HLH attended between 2004 and 2016. They were classified according to their probable cause in: associated with immunosuppression, cancer, post-infectious or idiopathic. Kaplan-Meier survival analysis was performed. Results: Twenty seven patients with HLH aged 18 to 87 years (59% men), were detected. Fourteen (52%) were secondary to immunosuppression, six (22%) were post-infectious, five (18%) were associated with cancer and two (7%) were of unknown cause. There were no significant differences in clinical or laboratory features between these etiologies. Within the immunosuppressed group, 12 (86%) were patients with oncologic or hematologic diseases or bone marrow transplantation. Associated cancers were mostly oncohematologic diseases. Thirty-day mortality was 53.4% (95% confidence intervals (CI) 32.7-70.3%), despite the treatment. Mortality was significantly associated with the presence of renal failure with a hazard ratio (HR) of 3.4 (95% CI of 1.2-9.9, p =0.025). Treatment of the underlying disease proved to be protective against mortality with an HR of 0.3 (95% CI 0.1 to 0.98, p = 0.046). Conclusions: The prognosis of HLH could be related to the treatment of the underlying disease. The study of the pathophysiology of this syndrome will allow a better understanding and treatment.
author	Warley,Fernando Bonella,Belén M Odstrcil-Bobillo,M. Silvina Otero,Victoria Waisman,Gabriel Bendelman,Gisela Giunta,Diego Peuchot,Verónica Ungaro,Catalina M
author_facet	Warley,Fernando Bonella,Belén M Odstrcil-Bobillo,M. Silvina Otero,Victoria Waisman,Gabriel Bendelman,Gisela Giunta,Diego Peuchot,Verónica Ungaro,Catalina M
author_sort	Warley,Fernando
title	Características clínicas y mortalidad de pacientes adultos con síndrome hemafagocítico, estudio de cohorte retrospectiva
title_short	Características clínicas y mortalidad de pacientes adultos con síndrome hemafagocítico, estudio de cohorte retrospectiva
title_full	Características clínicas y mortalidad de pacientes adultos con síndrome hemafagocítico, estudio de cohorte retrospectiva
title_fullStr	Características clínicas y mortalidad de pacientes adultos con síndrome hemafagocítico, estudio de cohorte retrospectiva
title_full_unstemmed	Características clínicas y mortalidad de pacientes adultos con síndrome hemafagocítico, estudio de cohorte retrospectiva
title_sort	características clínicas y mortalidad de pacientes adultos con síndrome hemafagocítico, estudio de cohorte retrospectiva
publisher	Sociedad Médica de Santiago
publishDate	2017
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872017000300008
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Características clínicas y mortalidad de pacientes adultos con síndrome hemafagocítico, estudio de cohorte retrospectiva

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