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Inmunoglobulina intravenosa en síndrome de activación macrofágica asociado a lupus eritematoso sistémico

Sergio Alberto Mendoza Álvarez, Roberto Galindo López, Daniela Sánchez Escalona, Yuridia Velázquez Santiago, Ana Sofía Sánchez Rodríguez

Resumen


Introducción: La linfohistiocitosis hemofagocítica (HLH), es una entidad clínica de evolución potencialmente fatal, caracterizada por la activación de macrófagos e histiocitos con hemofagocitosis en médula ósea y otros sistemas reticuloendoteliales, desencadenado por un defecto en el linfocito T al estimular la producción de interleucinas IL-1β, IL-6, interferón-γ (INF-γ) y factor de necrosis tumoral-α (TNF-α) que promueven la activación del macrófago. Esta afección cursa con fiebre, citopenias, esplenomegalia, hemofagocitosis en médula ósea, hipertrigliceridemia e hipofibrinogenemia, en el contexto de una enfermedad infecciosa, neoplásica o autoinmune. Casos Clínicos: Se presentan 2 casos de lupus eritematoso sistémico (LES) con síndrome de activación macrofágica (SAM), uno asociado a vasculitis del sistema nervioso central y otro con neutropenia febril, ambos con infección bacteriana agregada. El objetivo de este artículo es describir la utilidad de la inmunoglobulina intravenosa (IgIV) en pacientes que no pueden recibir tratamiento inmunosupresor. Conclusión: El diagnóstico de SAM en pacientes con actividad lúpica se debe sospechar por fiebre, citopenias, visceromegalias, hipertrigliceridemia e hipofibrinogenemia, un diagnóstico y tratamiento oportunos son importantes para disminuir de manera considerable la mortalidad. Se propone que el tratamiento de primera línea en paciente con LES asociado a SAM y sepsis, sea IgIV y como segunda línea inmunosupresores con esteroide intravenoso.


Palabras clave


Linfohistiocitosis Hemofagocítica; Síndrome de Activación Macrofágica; Lupus Eritematoso Sistémico

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Referencias


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