Linfohistiocitosis hemofagocítica: análisis de 18 casos. [artículo]
Por: Hernández Jiménez, María del Pilar [Medicina Interna]
| Díaz Pedroche, María del Carmen [Medicina Interna] | Laureiro Gonzalo, Jaime [Medicina Interna] | Madrid Pascual, Olaya [Medicina Interna] | Martín Clavero, Estela [Hematología y Hemoterapia] | Lumbreras Bermejo, Carlos [Medicina Interna].
Colaborador(es): Servicio de Medicina Interna | Servicio de Hematología y Hemoterapia.
Tipo de material: 
ArtículoEditor: Medicina clínica, 2016Descripción: 147(11):495-8.Recursos en línea: Solicitar documento Resumen: Background and objective: Hemophagocytic lymphohistiocytosis (HLH) is a serious condition, caused by an improper regulation of the immune response to different stimuli of the immune system. Early diagnosis and treatment are a challenge for the clinician.
Patients and method: We conducted a retrospective study at our institution between 2010 and 2015, of adult patients diagnosed with HLH, in accordance with the criteria of the Histiocyte Society, analyzing their clinical characteristics, diagnostic and etiological studies and the outcome.
Results: Eighteen patients were analyzed. Median time to diagnosis was 24 days. We found neoplastic etiology in 8 cases (7 hematologic), while it was infection-related in 6 (4 visceral leishmaniasis), and an inflammatory disease in one. In the remaining 3, an underlying cause for the HLH was not found. Course of treatment was corticosteroids in 16 patients, associated with cyclosporine in 2 of them, one received immunoglobulins, while another received etoposide with tacrolimus.
Conclusions: We emphasize the scarce use of etoposide therapy, the currently recommended treatment. Overall mortality was 44%, mainly associated with neoplastic etiology (67 compared to 16.6% mortality in infection-related etiology, P<.05).
| Tipo de ítem | Ubicación actual | Signatura | Estado | Fecha de vencimiento |
|---|---|---|---|---|
Artículo
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PC17872 (Navegar estantería) | Disponible |
Navegando Hospital Universitario 12 de Octubre Estantes Cerrar el navegador de estanterías
Formato Vancouver:
Hernández Jiménez P, Díaz Pedroche C, Laureiro J, Madrid O, Martín E, Lumbreras C. Linfohistiocitosis hemofagocítica: análisis de 18 casos. Med Clin (Barc). 2016 Dec 2;147(11):495-8.
PMID: 27726848
Contiene 10 referencias
Background and objective: Hemophagocytic lymphohistiocytosis (HLH) is a serious condition, caused by an improper regulation of the immune response to different stimuli of the immune system. Early diagnosis and treatment are a challenge for the clinician.
Patients and method: We conducted a retrospective study at our institution between 2010 and 2015, of adult patients diagnosed with HLH, in accordance with the criteria of the Histiocyte Society, analyzing their clinical characteristics, diagnostic and etiological studies and the outcome.
Results: Eighteen patients were analyzed. Median time to diagnosis was 24 days. We found neoplastic etiology in 8 cases (7 hematologic), while it was infection-related in 6 (4 visceral leishmaniasis), and an inflammatory disease in one. In the remaining 3, an underlying cause for the HLH was not found. Course of treatment was corticosteroids in 16 patients, associated with cyclosporine in 2 of them, one received immunoglobulins, while another received etoposide with tacrolimus.
Conclusions: We emphasize the scarce use of etoposide therapy, the currently recommended treatment. Overall mortality was 44%, mainly associated with neoplastic etiology (67 compared to 16.6% mortality in infection-related etiology, P<.05).
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