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2013, Número 4

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Gac Med Mex 2013; 149 (4)


Síndrome hemofagocítico. Conceptos actuales

Espinosa BKA, Garciadiego FP, León RE

Idioma: Español
Referencias bibliográficas: 41
Paginas: 431-457
Archivo PDF: 97.56 Kb.


RESUMEN

La linfohistiocitosis hemofagocítica es un síndrome que se caracteriza por activación inmune patológica que puede presentarse de forma primaria (asociada a mutaciones genéticas) o relacionado a infecciones, neoplasias o enfermedades autoinmunes. El cuadro clínico se caracteriza por una inflamación desproporcionada que produce fiebre, citopenias, esplenomegalia, hemofagocitosis en médula ósea, hipertrigliceridemia e hipofibrinogenemia. La mortalidad relacionada con el síndrome es alta, por lo que se debe mantener un alto índice de sospecha e iniciar de forma temprana el tratamiento con inmunoquimioterapia y trasplante de médula ósea en casos primarios o refractarios. En este artículo realizamos una revisión de las manifestaciones clínicas, patología, diagnóstico y tratamiento de estos pacientes.


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