RESUMO
Abstract Background: Chronic active EBV infection (CAEBV) of T-cell or NK-cell type is an EBV+ polyclonal, oligoclonal or often monoclonal lymphoproliferative disorder (LPD) recognized as representing the spectrum of EBV-associated T-cell and NK-cell LPD with different clinical presentations; one systemic and two cutaneous disorders including hydroa vacciniforme-like T-cell LPD and mosquito bite hypersensitivity. The systemic form of the disease is characterized by fever, persistent hepatitis, hepatosplenomegaly and lymphadenopathy, which shows varying degrees of clinical severity depending on the immune response of the host and the EBV viral load. Case reports: We described the clinicopathological findings of two children with CAEBV with a brief review of the literature. Conclusions: Recognition of the disease is important for adequate management of the patient. EBV analysis should be included in the principal diagnostic tests for febrile children.
Resumen Introducción: La infección crónica activa (CA) de células T o células tipo NK por virus de Epstein-Barr (VEB) es un desorden linfoproliferativo (DLP) VEB+ policlonal, oligoclonal o, frecuentemente, monoclonal reconocido como representación del espectro del DLP de células T y células NK asociado con VEB que tiene diversas presentaciones clínicas: un padecimiento sistémico y dos cutáneos que incluyen el DLP de células T que semeja hidroa vacciniforme y la hipersensibilidad por picadura de mosquito. Los síntomas de la enfermedad sistémica incluyen fiebre, hepatitis persistente, hepatoesplenomegalia y linfadenopatías que muestran diferente grado de severidad clínica, dependiendo de la respuesta inmune del hospedero y de la carga viral del VEB. Casos clínicos: Se describen los hallazgos clínico-patológicos de dos niños con CAVEB y una breve revisión de la literatura. Conclusiones: Es importante reconocer esta enfermedad para proporcionar el manejo adecuado al paciente. El análisis de VEB debería incluirse como una de las principales pruebas diagnósticas en niños con fiebre.
RESUMO
Severe chronic active Epstein-Barr virus (EBV) infection is a rare and life-threatening illness. Although the criteria for diagnosis include chronic or recurrent infectious mononucleosis-like symptoms lasting more than 6 months and high titers of anti-EBV antibodies, clinical and laboratory findings may be heterogeneous and flexible application of those criteria is necessary in cases showing typical clinical and pathologic findings. We report a case of severe chronic active EBV infection in a 62-yr-old female patient who showed classical clinical findings with infiltration of EBV-infected T lymphocytes in the bone marrow, spleen, and lymph nodes, and died four months after presentation.