Subject(s)
Papilloma, Choroid Plexus/diagnosis , Humans , Infant , Magnetic Resonance Imaging , Meninges/pathologyABSTRACT
No disponible
Subject(s)
Humans , Female , Infant , Papilloma, Choroid Plexus , Vomiting/etiology , Cerebrospinal Fluid/cytology , Cerebrospinal Fluid , Magnetic Resonance Imaging/methods , Gadolinium , Calcinosis/complications , Calcinosis , Papilloma, Choroid Plexus/complications , Papilloma, Choroid Plexus/diagnosis , Papilloma, Choroid Plexus/therapy , Ultrasonography/trends , Hydrocephalus/complications , Diagnosis, DifferentialABSTRACT
La linfohistiocitosis hemofagocítica (LHH) es un síndrome clínico raro, posiblemente infradiagnosticado, más frecuente en niños. Pueden ser cuadros agresivos y evolucionar en poco tiempo a fallo multiorgánico letal. Simula cuadros de sepsis infecciosa, aunque con peor respuesta y evolución. Se debe sospechar ante un niño pequeño con fiebre persistente de origen desconocido, afectación del estado general, hepatoesplenomegalia, citopenias, aumento de triglicéridos y ferritina y disminución del fibrinógeno. En la resonancia magnética cerebral se puede apreciar un realce difuso leptomeníngeo y perivascular, áreas parcheadas hiperintensas en T2 en sustancia blanca de ambos hemisferios cerebrales y atrofia cerebral. La secuencia de difusión es útil en la estadificación de las lesiones. Presentamos un caso clínico de LHH familiar de evolución mortal y realizamos una revisión bibliográfica de las características clínicas, anatomopatológicas y radiológicas de esta entidad (AU)
Hemophagocytic lymphohistiocytosis (HL) is a rare syndrome, although more common in children, that may be underdiagnosed. The clinical presentation can be aggressive, and patients may rapidly develop lethal multiple organ failure. HL simulates the presentation of infectious sepsis, although the response to treatment and evolution are worse. HL should be suspected in young children with persistent fever of unknown origin, general malaise, hepatosplenomegaly, cytopenia, elevated triglycerides and ferritin, and decreased fibrinogen. Brain MRI shows diffuse leptomeningeal and perivascular enhancement, patchy areas of hyperintensity in the white matter of both cerebral hemispheres on T2-weighted sequences, and cerebral atrophy. Diffusion-weighted sequences are useful for staging the lesions. We present a fatal case of familial HL and review the literature about the clinical, histological, and radiological characteristics of this disease (AU)
Subject(s)
Humans , Female , Child , Lymphohistiocytosis, Hemophagocytic/complications , Lymphohistiocytosis, Hemophagocytic , Splenomegaly/complications , Splenomegaly , Leukopenia/complications , Leukopenia , Magnetic Resonance Imaging/methods , Thrombocytopenia , Biopsy , Phagocytosis , SkullABSTRACT
Hemophagocytic lymphohistiocytosis (HL) is a rare syndrome, although more common in children, that may be underdiagnosed. The clinical presentation can be aggressive, and patients may rapidly develop lethal multiple organ failure....HL simulates the presentation of infectious sepsis, although the response to treatment and evolution are worse. HL should be suspected in young children with persistent fever of unknown origin, general malaise, hepatosplenomegaly, cytopenia, elevated triglycerides and ferritin, and decreased fibrinogen. Brain MRI shows diffuse leptomeningeal and perivascular enhancement, patchy areas of hyperintensity in the white matter of both cerebral hemispheres on T2-weighted sequences, and cerebral atrophy. Diffusion-weighted sequences are useful for staging the lesions. We present a fatal case of familial HL and review the literature about the clinical, histological, and radiological characteristics of this disease.
