[Acute hemolytic crisis followed by fulminant hepatic failure with fatal outcome, as a first clinical manifestation of Wilson's disease]. / Crise hemolítica aguda seguida por insuficiência hepática fulminante com evolução fatal, como primeira manifestação clínica da doença de Wilson.

We describe in this work a clinical case of a female patient aged 21 years, bearer of Wilson's disease, a first clinical manifestation of the disease occurred as an acute hemolytic crisis followed by fulminant hepatic failure evolving to death after 26 days' internment. The definitive diagnosis was obtained only as a quantitative measurement of hepatic copper from the necropsy material. The search for Kayser-Fleischer ring was negative and the serum ceruloplasmin level was 9 mg/dl (15 to 60). No involvement of the central nervous system was noted from the pathologic analysis. The patient presented two Coombs negative hemolytic crises during the internment; the first on being admitted to hospital and the second after a transjugular hepatic biopsy carried out on the 16th day after internment. The last hemolytic crisis was accompanied by an increase of serum and urinary copper levels. On this occasion the patient evolved to a progressive hepatic failure with severe jaundice and hepatic encephalopathy. We are presenting the clinical-biochemical evolution of the patient and we shall discuss the existent hypotheses to the pathophysiology of this rare form for manifestation of the Wilson's disease as well the diagnostic difficulties.

[Two cases of severe cutaneous vasculitis with thrombocytopenia associated with hepatic cirrhosis: autoimmune and infective-inflammatory component with endothelial lesion and restrictive pulmonary pattern in one case]. / Dois casos de vasculite cutânea grave com plaquetopenia associada à cirrose hepática: componente autoimune e infecto-inflamatório com lesão endotelial e padrão restritivo pulmonar em um caso.

Two clinical cases of female patients with hepatic cirrhosis and autoimmune multisystemic involvement with infectious intercurrent are reported. Case 1 presented infective endocarditis and erysipelas on the left thigh. In the course of the clinical picture a cutaneous vasculitis developed in the same place together with autoimmune thrombocytopenia, leukopenia and pulmonary restrictive picture with inflammatory pattern. There are also elevate immune complexes and complement consumption. Case 2 presented erysipelas on the left thigh cutaneous vasculitis and complement consumption. In Case 1 the infective endocarditis was treated with antibiotic therapy during 4 weeks followed by 1 mg/kg corticoid (Prednisone) with thrombocytopenia and leukopenia reversion. Case 2 presented an improvement with antibiotic only. The relation between chronic liver diseases and systemic autoimmune phenomena is commented, special attention being paid to the cutaneous, hematological and pulmonary affection.

Amiloidose e plasmocitoma secretor de cadeia leve lambda: / Amyloidosis and light lambda chain secreting plasmacytoma

Apresenta-se o estudo de um caso de amiloidose de multiplas visceras, sem evidencias clinicas, radiologicas e laboratoriais de associacao a outras entidades, levando a obito devido ao macico acometimento cardiaco. A autopsia todavia, revelou plasmocitoma em oitavo arco costal direito, caracterizado a imuno-histoquimica (metodo PAP de imunoperoxidase) como secretor de cadeia leve lambda. Os autores, baseados nesta analise, sugerem que nos casos de amiloidose sem sinais de outras molestias associadas, sejam realizadas biopsias de medula ossea, eventualmente em varias localizacoes, e que, em todas as proliferacoes plasmocitarias sejam aplicados metodos imuno-histoquimicos para pesquisa de cadeias leves e pesadas de imunoglobulinas, favorecendo a diferenciacao de proliferacoes reacionais (policlonais) das neoplasicas (monoclonais)

Doenca da cadeia alfa. / Alpha chain disease

A doenca da cadeia alfa se caracteriza por uma infiltracao linfoplasmocitaria no intestino delgado e ganglios mesentericos.Os autores descrevem um caso e salientam a importancia do diagnostico precoce, assim como o valor da investigacao do intestino delgado pela cirurgia, com a finalidade de se obter um melhor estadiamento da molestia. Nos casos em que a infiltracao linfoplasmocitaria e restrita a mucosa, o uso de antibioticos provoca remissao da doenca por tempo prolongado. Quando a invasao atinge camadas mais profundas, como sub-mucosa e muscular, deve-se considerar a doenca como um verdadeiro linfoma, usando medicacao apropriada