ABSTRACT
Varón de 38 años que acude por un cuadro de dos meses de evolución caracterizado por síndrome constitucional, fiebre, poliadenopatías y hepatoesplenomegalia. Además indicó cefalea, visión borrosa bilateral y miodesopsias. La agudeza visual corregida fue de 20/50 y 20/200. El segmento anterior no mostraba alteración. El fondo de ojo evidenció discos sobreelevados y borrados, acompañados de desprendimiento seroso del neuroepitelio mayor en el ojo izquierdo. Los estudios de ambos ojos mostraron un campo visual con aumento de la mancha ciega; la angiografía fluoresceínica mostró hiperfluorescencia tardía discal; el electrorretinograma evidenció compromiso de conos y bastones; el potencial visual detectó alteración en la percepción y la conducción de estímulos. Los estudios de imagen fueron esencialmente normales. Durante su hospitalización presentó paraparesia, insuficiencia renal, endocrinopatía, alteraciones dérmicas y lesiones osteoblásticas con biopsias negativas. Se documentó gammapatía monoclonal, planteándose el diagnóstico de síndrome de POEMS -Polineuropatía, Organomegalia, Endocrinopatía, Gammapatía Monoclonal y alteraciones dermatológicas e iniciándose quimioterapia con dexametasona/melfalán, con buena respuesta a los 3 meses
The cases is presented of a 38 year-old male with a constitutional syndrome, fever, multiple swollen lymph nodes, and hepatosplenomegaly of 2 months onset. There was also mention of headache, bilateral blurred vision, and myiodesopsias. Best correct visual acuity was 20/50 and 20/200. The anterior segment was unremarkable. The fundus of both eyes showed raised and erased discs, accompanied by serous detachment, greater in the left eye. Complementary studies of both eyes showed a visual field with increased blind spot; fluorescein angiography indicated late disc hyperfluorescence. The electroretinogram showed compromise of cones and rods, and the visual evoked potential detected alteration in the perception and conduction of stimuli. The imaging studies were essentially normal. During his hospital admission he presented with paraparesis, renal failure, endocrinopathy, skin alterations, and osteoblastic lesions with negative biopsies. Monoclonal gammopathy was documented, and the diagnosis of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes) syndrome was made. The chemotherapy started with dexamethasone/melphalan, with a good response at 3 months
Subject(s)
Humans , Male , Adult , Eye Diseases/etiology , Nervous System Diseases/etiology , POEMS Syndrome/complications , POEMS Syndrome/diagnosisABSTRACT
The cases is presented of a 38 year-old male with a constitutional syndrome, fever, multiple swollen lymph nodes, and hepatosplenomegaly of 2 months onset. There was also mention of headache, bilateral blurred vision, and myiodesopsias. Best correct visual acuity was 20/50 and 20/200. The anterior segment was unremarkable. The fundus of both eyes showed raised and erased discs, accompanied by serous detachment, greater in the left eye. Complementary studies of both eyes showed a visual field with increased blind spot; fluorescein angiography indicated late disc hyperfluorescence. The electroretinogram showed compromise of cones and rods, and the visual evoked potential detected alteration in the perception and conduction of stimuli. The imaging studies were essentially normal. During his hospital admission he presented with paraparesis, renal failure, endocrinopathy, skin alterations, and osteoblastic lesions with negative biopsies. Monoclonal gammopathy was documented, and the diagnosis of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes) syndrome was made. The chemotherapy started with dexamethasone/melphalan, with a good response at 3 months.
