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

Marzo_2017

artículo de investigación 344 Rev Med Chile 2017; 145: 344-350 Características clínicas y mortalidad de pacientes adultos con síndrome hemafagocítico, estudio de cohorte retrospectiva Fernando Warley1,3, Belén M. Bonella1,3, M. Silvina Odstrcil-Bobillo1,3, Victoria Otero2, Gabriel Waisman1, Gisela Bendelman1,3, Diego Giunta3, Verónica Peuchot3, Catalina M. Ungaro1,3 Hemophagocytic lymphohistiocytosis. Experience in 27 patients 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. (Rev Med Chile 2017; 145: 344-350) Key words: Hemaphagocytic;  Immunosuppression; Immunocompromised Host; Lymphohistiocytosis. 1Departamento de Medicina Interna, Hospital Italiano de Buenos Aires, Argentina. 2Sección Hematología, departamento de Medicina Interna, Hospital Italiano de Buenos Aires, Argentina. 3Área de Investigación en Medicina Interna, departamento de Medicina Interna, Hospital Italiano de Buenos Aires, Argentina. Conflictos de interés: Los autores refieren no presentar conflictos de interés que declarar. Recibido el 24 de agosto de 2016, aceptado el 6 de marzo de 2017. Corresponenicia a: Dr. Warley Fernando Hospital Italiano de Buenos Aires, Tte. Gral. J. D. Perón 4190, Buenos Aires, Argentina. ferwarley@gmail.com La linfohistiocitosis hemofagocítica o síndrome hemofagocítico (SHF) se caracteriza por la activación del sistema inmunológico de manera exacerbada e inefectiva. Una de sus principales componentes es la infiltración de linfocitos y macrófagos con actividad hemofagocítica en diferentes órganos, especialmente el hígado, bazo, médula ósea y sistema nervioso central1. El SHF adquirido puede ser secundario a infecciones virales, enfermedades malignas, entidades autoinmunes, alteraciones metabólicas, inmunodeficiencias adquiridas o inmunosupresión asociada


Marzo_2017
To see the actual publication please follow the link above