[Kaposi's disease. Form with lymph node manifestations in a young African. Difficulty of the initial diagnosis and relation to dysimmune lymphadenitis].
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Biomedical subjects
Publications and source records attributed to Y Pinaudeau.
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A 44-year-old woman who had been taking oral contraceptives for 10 yr developed abdominal pain and abnormalities of liver tests, which led to the discovery of multiple filling defects on liver scan and of large hepatic zones of decreased density on computerized tomography of the abdomen. These abnormalities were due to sharply demarcated areas of hepatic multilobular hemorrhagic necrosis, associated with marked changes of liver arterioles and small hepatic veins. Since oral contraceptives have been incriminated in the genesis of hepatic vascular alterations, the possible relationship between focal hemorrhagic necrosis of the liver and these agents is suggested.
In a 75-year-old man, the rapid development of a pancytopenia as a result to total marrow failure, in the absence of tumour or extramedullary myelopoiesis, but with a histological appearance of the marrow identical to that seen in agnogenic myeloid metaplasia led to a diagnosis of malignant myelofibrosis. The patient died 7 months after the apparent onset of the disease. The 48 other published cases are discussed. Only 17 are considered to be true cases of malignant myelofibrosis.
Regencrative nodular hyperplasia (RHN) is a rare condition, the diagnosis of which is based upon histological findings. It is seen in Felty's syndrome with portal hypertension (PHT), as was the case in the patient reported here. This was a 72-year-old man, with long standing rheumatoid arthritis, hepatosplenomegaly, a neutrophil leucopaenia and oesophageal varices responsible for recurrent haematemeses. Despite a portocaval anastomosis, the patient died from postoperative acute hepatic failure. Histological study revealed changes in the hepatocytes and the reticulin system typical of RNH without cirrhosis. The relationship between Felty's syndrome and RHN, as well as the mechanism of the hypertension, are discussed in the light of cases from the literature.
Malignant sarcoma reaching a focal endometriosis is a rare condition. It cannot be distinguished from an endometrial sarcoma except in cases which are developped in extra-uterine endometriosis without uterus involvement. The case reported is a forty-seven old white woman with a sarcoma of the endometrial stroma reaching a Douglas focal endometriosis. Cases from literature are reviewed.
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The authors report a case of Woringer-Kolopp syndrome. This was a rare form with multiple cutaneous features with a typical clinical and histological appearance. Ultrastructural study revealed the presence of granules with an opaque centre in the cytoplasm of the abnormal cells, and the close relationship of the latter with neurites. On the basis of these characteristics, they may be likened to the Merkel cells of the epidermis. Thus Woringer-Kolopp disease could be related to the proliferation of Merkel cells.
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