Case report. Portal cavernomatosis as presentation of POEMS syndrome
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Sociedad Argentina de Gastroenterología
2018
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| _version_ | 1876459293129048064 |
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| author | Emanuel José Saad |
| author_facet | Emanuel José Saad |
| contents | Case report. Portal cavernomatosis as presentation of POEMS syndrome Emanuel José Saad María Victoria Mulqui Candelaria Mendoza Juliana Mandrile Domingo Cesar Balderramo Medicina POEMS syndrome polyneuropathies paraproteinemias venous thrombosis POEMS syndrome is a rare paraneoplastic disorder with an underlying plasma cell dyscrasia associated with peripheral polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy and skin changes. While, ascites is a common sign in patients with POEMS, it is unfrequently associated with portal cavernomatosis and portal hypertension. We report a case of a 41-year-old man who presented with asthenia, weight loss and ascites. There was diagnosed portal cavernomatosis and the ascitic fluid exam was compatible with transudate. Later, the patient developed polyneuropathy and cutaneous hyperpigmentation, and there were demonstrated monoclonal component IgA in serum, Castleman disease in an inguinal lymphadenophaty and osteosclerotic injuries in bones, and the patient was diagnosed with POEMS syndrome. Only 5 cases of POEMS syndrome with portal hypertension have been reported. Nevertheles, in anyone of them portal thrombosis was demonstrated, and there was described only one case of a patient with POEMS syndrome, who developed portal thrombosis. 2018 artículo científico 2469-1119 https://www.redalyc.org/articulo.oa?id=199359103012 https://www.redalyc.org/journal/1993/199359103012/ https://www.redalyc.org/journal/1993/199359103012/html/ https://www.redalyc.org/journal/1993/199359103012/199359103012.epub https://www.redalyc.org/journal/1993/199359103012/movil en http://www.redalyc.org/revista.oa?id=1993 Acta Gastroenterológica Latinoamericana application/pdf Sociedad Argentina de Gastroenterología Acta Gastroenterológica Latinoamericana (Argentina) Num.4 Vol.48 |
| format | Artículo científico |
| id | redalyc_199359103012 |
| institution | Redalyc |
| language | en |
| publishDate | 2018 |
| publisher | Sociedad Argentina de Gastroenterología |
| spellingShingle | Case report. Portal cavernomatosis as presentation of POEMS syndrome Emanuel José Saad Medicina POEMS syndrome polyneuropathies paraproteinemias venous thrombosis Case report. Portal cavernomatosis as presentation of POEMS syndrome Emanuel José Saad María Victoria Mulqui Candelaria Mendoza Juliana Mandrile Domingo Cesar Balderramo Medicina POEMS syndrome polyneuropathies paraproteinemias venous thrombosis POEMS syndrome is a rare paraneoplastic disorder with an underlying plasma cell dyscrasia associated with peripheral polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy and skin changes. While, ascites is a common sign in patients with POEMS, it is unfrequently associated with portal cavernomatosis and portal hypertension. We report a case of a 41-year-old man who presented with asthenia, weight loss and ascites. There was diagnosed portal cavernomatosis and the ascitic fluid exam was compatible with transudate. Later, the patient developed polyneuropathy and cutaneous hyperpigmentation, and there were demonstrated monoclonal component IgA in serum, Castleman disease in an inguinal lymphadenophaty and osteosclerotic injuries in bones, and the patient was diagnosed with POEMS syndrome. Only 5 cases of POEMS syndrome with portal hypertension have been reported. Nevertheles, in anyone of them portal thrombosis was demonstrated, and there was described only one case of a patient with POEMS syndrome, who developed portal thrombosis. 2018 artículo científico 2469-1119 https://www.redalyc.org/articulo.oa?id=199359103012 https://www.redalyc.org/journal/1993/199359103012/ https://www.redalyc.org/journal/1993/199359103012/html/ https://www.redalyc.org/journal/1993/199359103012/199359103012.epub https://www.redalyc.org/journal/1993/199359103012/movil en http://www.redalyc.org/revista.oa?id=1993 Acta Gastroenterológica Latinoamericana application/pdf Sociedad Argentina de Gastroenterología Acta Gastroenterológica Latinoamericana (Argentina) Num.4 Vol.48 |
| title | Case report. Portal cavernomatosis as presentation of POEMS syndrome |
| topic | Medicina POEMS syndrome polyneuropathies paraproteinemias venous thrombosis |
| url | https://www.redalyc.org/articulo.oa?id=199359103012 https://www.redalyc.org/journal/1993/199359103012/ https://www.redalyc.org/journal/1993/199359103012/html/ https://www.redalyc.org/journal/1993/199359103012/199359103012.epub https://www.redalyc.org/journal/1993/199359103012/movil |