Utilize este identificador para referenciar este registo: http://hdl.handle.net/10400.17/128
Título: Púrpura. Manifestação de Amiloidose Sistémica Primária
Autor: Lestre, S
Gonçalves, A
João, A
Ferreira, A
Apetato, M
Palavras-chave: Idoso
Amiloidose
Púrpura
Diagnóstico
Complicações
Data: 2009
Editora: Centro Editor e Livreiro da Ordem dos Médicos
Citação: Acta Med Port 2009; 22 (3): 307-312
Resumo: Primary Systemic Amyloidosis (AL) is the most frequent form of systemic amyloidosis and its morbilility is associated with immunoglobulin light chains deposition in vital organs. The mucocutaneous manifestations occur in about 30-40% of the cases and are important in diagnostic suspicion, once they appear in early stages of disease. We report a 71-years-old female patient, with disseminated purpura and cutaneous fragility with 6 months of evolution, accompanied by recent complaints of dysphagy. The first laboratory evaluation didn't show any alterations. The histological and immunohistochemical study of subcutaneous abdominal fat and skin biopsy showed lambda type amyloid protein. In the systemic work-up, we highlight a proteinúria > 1g/24h with Bence Jones proteins and the presence of monoclonal immunoglobulin light chain (lambda type) in serum immunoelectrophoresis. With the diagnosis of primary systemic amyloidosis, treatment with prednisolone and melphalan was started.
URI: http://hdl.handle.net/10400.17/128
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MED - Artigos

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