Tabla de Contenidos > Volumen 17 número 4 - Diciembre 2020
ISSN 0718-0918
Tabla de Contenidos > Actualización
LINFOHISTIOCITOSIS HEMOFAGOCÍTICA SECUNDARIA A INFECCIÓN CRÓNICA ACTIVA POR VIRUS DE EPSTEIN-BARR EN UN HOSPITAL PEDIÁTRICO EN MÉXICO. SERIES DE CASOS Y REVISIÓN DE LA LITERATURA
Yadira Janet González Paredes, Mara Núñez Toscano, Gabriela Tavera Rodríguez, Lourdes González Pedroza, Angélica Monsiváis Orozco, Norma López Santiago, Rogelio Paredes Aguilera
Departamento de Hematología Pediátrica, Instituto Nacional de Pediatría, Ciudad de México, México.

Resumen | Abstract | Texto completo HTML | Descargar cuerpo en pdf

Introduction: Secondary hemophagocytic lymphohistiocytosis (HLH) is associated with malignant, genetic or autoimmune diseases but also with infections mainly EBV in up to 70%, however there is little information. This entity is characterized by a variable and recurrent course that leads to high morbidty and mortality with life-threatening complications.

Objective: To describe the clinical characteristics and evolution of pediatric patients with a diagnosis of HLH secondary to CAEBV. Results: 7 patients were included, mean age at diagnosis was 52 months with a predilection for males. All patients were treated with a multiagent chemotherapeutic regimen, including corticosteroid, etoposide, and cyclosporine. After treatment, 6 patients presented remission and one of them had reactivation. The mean follow-up was 19 months and disease-free survival (DFS) 16 months.

Conclusion: We can observe that the clinical course is variable, sometimes fulminant and with poor response to treatment. An early diagnosis as well as detecting prognostic factors could help to adapt treatment strategies that would change the clinical course.

Key words: chronic active Epstein-Barr virus infection, hemophagocytic lymphohistiocytosis, children

Revista Pediatría Electrónica
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Correo electrónico: gmedina.uchile@gmail.com
ISSN 0718-0918