| Journal: | Revista médica de Chile |
| Database: | PERIÓDICA |
| System number: | 000407082 |
| ISSN: | 0034-9887 |
| Authors: | Warley, Fernando1 Bonella, Belén M1 Odstrcil Bobillo, M. Silvina1 Otero, Victoria1 Waisman, Gabriel1 Bendelman, Gisela1 Giunta, Diego1 Peuchot, Verónica1 Ungaro, Catalina M1 |
| Institutions: | 1Hospital Italiano de Buenos Aires, Departamento de Medicina Interna, Buenos Aires. Argentina |
| Year: | 2017 |
| Season: | Mar |
| Volumen: | 145 |
| Number: | 3 |
| Pages: | 344-350 |
| Country: | Chile |
| Language: | Español |
| Document type: | Artículo |
| Approach: | Analítico, descriptivo |
| English abstract | 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 |
| Disciplines: | Medicina |
| Keyword: | Hematología, Inmunología, Linfohistiocitosis hemofagocítica, Inmunosupresión, Características clínicas, Mortalidad |
| Keyword: | Medicine, Hematology, Immunology, Hemophagocytic lymphohistiocytosis, Immunosuppression, Clinical characteristics, Mortality |
| Full text: | Texto completo (Ver HTML) |
