[Gougerot-Sjögren syndrome, angioimmunoblastic lymphadenopathy and later immunoblastic sarcoma].
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Biomedical subjects
Publications and source records attributed to G Lemaigre.
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The authors report the case of a 62-year-old woman in whom, 18 months following the development of bilateral parotid hypertrophy followed by a dry syndrome, there was the onset of an angioimmunoblastic lymphadenopathy (AIL) which consisted of the association of a multiple lymphadenopathy with a characteristic histological appearance, although poor in plasmocytes, and a febrile syndrome, but in the absence of a complete syndrom from a laboratory standpoint. This fact, incombination with the richness of the nodes in epitheloid cells, is such that this case resembles the type II "dysimmune and pseudo-lymphomatous lymphadenopathies" (DPLL) of Delsol et al. Although the term AIL has never been used before in the title of previous publications of pseudo-lymphomas occuring during Sjögren's syndrome, it would seem possible, as has already been suggested by Diebold et al. (3) with regard to several cases, that certain of these pseudo-lymphomas are true AIL. The rapidly fatal course with visceral spread shown at autopsy and above all the presence of exclusively immunoblastic plaques in several mode areas led, on the basis of the criteria of Nathwani et al. (22), to acceptance of the coexistence of an immunoblastic sarcoma.
Nine hypoglycaemia-inducing pancreatic tumours were studied by electron microscopy. In 8 of these tumours, it was possible to identify, within the cell cytoplasm, secretory granules with a "paracrystalline" content, identical in appearance to the granules of the B cell of the normal human pancreas. Thus electron microscopy would appear to be a reliable and sensitive method for the morphological identification of these endocrine tumours of the pancreas.
Africa, and in particular North Africa, is a site of high prevalence of carcinoma of the nasopharynx in the young subject. There is a marked male predominance and a clear peak of incidence between 15 and 25 years. The presenting feature is usually rapidly growing cervical lymphadenopathy. The diagnosis is based upon histological examination of these nodes. It is a squamous carcinoma, usually highly undifferentiated and difficult to identify. However, for the experienced observer, the special appearance of tumour proliferation makes it possible to localise the primary tumour in the epi-pharyngeal region. Current work is aimed at research into a possible genetic predisposition and the relationship which the carcinoma may have with the Epstein-Barr virus.
Eosinophilic ileitis is observed mainly in three coastal areas: Holland, Japan and Brittany. It presents in the form of a parietal inflammation, stenosing the intestinal lumen or a sessile tumour of limited volume. The symptoms are generally those of appendicitis but a past history of allergy and the discovery of eosinophilia greater than 10 p. cent may lead to the diagnosis of eosinophilic granuloma which only histology can confirm. Surgical treatment consists of segmental resection of the small intestine. The pathogenesis of the granuloma, which was for long attributed to the presence in the wall of the intestine of ascaris larvae, is at present debated. Are there non-parasitic eosinophilic granulomas? The case reported here is perhaps an example.
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The toxicity of the "glue thistle" is well known in Algeria. 10 children were involved in a case of collective poisoning. Only two survived. Histo-pathological studies were made in four cases, the clinical and histological picture being that of a fulminating hepatitis with major hepatocellular necrosis.
The authors report a comparative retrospective study of 124 end-to-side portal anastomoses and 63 side-to-side anastomoses. The influence of a certain number of clinical and pathological factors on mortality and immediate and long-term survival, were statistically verified. The quality of the functional result result was analysed in each group of operated patients. Comparison of the results of trunkular anastomoses with or without interruption of the portal vein, showed both as regards, post-operative and late mortality, that the functional results were definitely in favor of end-to-side anastomosis. Thus side-to-side anastomoses gave 40,4 p. 100 of good results and 59,6 average or poor results, as against 60 p. 100 gave rise to portal systemic encephalopathy in 17 p. 100 of cases as against 35 p. 100 in the case of the side-to-side anastomosis. Only the sub-group of operated patients with hepatic vein thrombosis seemed to benefit from side-to-side anastomosis.
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An anatomoclinical study is carried out in 11 cases of apparently primary intrahepatic cholestasis. Two groups have been distinguished: one includes three cases with poorly specific and disparate changes. Contrarely, the other group, including eigh cases, shows quite evocative and stereo-typed histological lesions. These are alwayst epithelial and segmental canalar lesions with characteristic cellular infiltrations of variable occurrence and location. The lesions develop into multilating sclerosis with progressive loss of the florid lesions. This group may be assimilated to the affection commonly known as Hanot and Mac Mahon's disease. Antimitochondria antibodies are very often found in this anatomoclinical entity but their absence does not allow exclusion of the diagnosis. Nevertheless, anatomical, clinical and biological correlation remains indispensable.
This case of recurrent migratory superficial phlebitis is reported because of the highly unusual nature of the lesion observed. The picture was dominated by periphlebitis with inflammatory granuloma, giant cells and elastophagia. When the condition had been present for sixteen months, an aortic arch syndrome developed in an inflammatory context. In this light, various auto-immunological etiologies were considered, among them Takayashu's arteritis of which, in the author's opinion, this would be the first case to be combined with venopathy.
Two cases of intestinal perforation due to necrotising enteritis in patients with pica are presented. The aetiopathogenesis of pica is discussed as well as its role in the development of necrotising enteritis.
Study of as homogeneous series as possible of 72 cases of gastrointestinal haemorrhage in patients with portal hypertension showed that the major cause of bleeding, accounting for almost 70% of cases, was ulceration of oesophageal varices. The presence at autopsy of haemorrhagic or necrotic lesions of the gastric mucosa is not felt to be a definite argument in favour of a gastric origin of the bleeding. Ulceration of gastric varices is considered to be an uncommon possibility, by virtue of the absence of dystrophic changes in the overlying mucosa, in contrast to the state of affairs seen in the oesophagus. Finally, the authors noted the relatively common presence of an associated malignant tumour (more than 8% of cases). This should perhaps lead to more profound investigations in order to seek a malignant intra- or extrahepatic malignant tumour before any bypass procedure to correct portal hypertension is undertaken.
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