Biomedical subjects
Publications and source records attributed to A Lavergne.
In a Caucasian woman with a history of ocular and pulmonary sarcoidosis, the occurrence of sclerosing peritonitis with exudative ascites but without any of the well-known causes of this syndrome prompts us to consider that sclerosing peritonitis is a manifestation of sarcoidosis. The dramatic improvement that occurred on corticosteroid therapy adds credibility to this previously unreported association.
We report 7 prospectively followed cases of lymphomatous polyposis of the gastrointestinal tract. They were characterized by multiple polypoid lesions affecting several segments of the gastrointestinal tract always involving the colon and the rectum. An ileocecal mass was present in 4 cases. Regional lymph node involvement was constant. Peripheral lymphadenopathy was frequent (5 cases out of 7), as was other extra-digestive extension to the bone marrow (4 cases out of 7) and cavum (3 cases out of 7). The histopathological aspect was that of a small cleaved cells (working formulation) or centrocytic (Kiel classification) non-Hodgkin's lymphoma. The peculiar morphology and phenotype of the tumoral B-lymphocytes suggest their possible follicle marginal zone origin. Lymphomatous polyposis bore a rapidly fatal prognosis in every case (mean survival 20 months). This study of seven patients together with the 20 well-documented cases of the literature confirms the existence of lymphomatous polyposis as a distinctive clinicopathological entity among gastrointestinal non-Hodgkin's lymphoma.
Ten cases of gastric mucosa-associated lymphoid-tissue B-cell lymphoma were studied on surgical specimens by histology and immunohistochemistry, with monoclonal and polyclonal antibodies for B- and T-cells. For the first time, percentage of centroblast-like cells was appreciated, using their LN1 positivity, by opposition to the negativity of centrocyte-like cells (LN2 immunoreactivity alone). Lymphomas were divided into four main groups: A) centrocyte-like cells; B) centrocyte-like cells and immunocytoma; C) centrocyte-like cells admixed to centroblast-like cells; and D) centrocyte-like cells, immunocytoma and at least 30% of centroblast-like cells. Group C was divided into 3 subgroups: C1 (rare centroblast-like cells); C2 (30-50% of centroblast-like cells); and C3 (predominant centroblast-like cells). Therefore low grade (A, B, C1) and high grade malignancy groups (C2, C3, D) were identified: this preliminary subdivision could be extended in larger series and applied to mucosa-associated lymphoid-tissue lymphoma from other sites. Furthermore, the possible prognostic significance of this subdivision could be evaluated by correlation with long term follow-up.
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We report the cases of two patients with coeliac disease in whom chronic diarrhoea persisted in spite of strict adherence to a gluten free-diet and regression of intestinal villous atrophy. Collagenous colitis was evidenced in both patients. Six cases of such association have already been described. Because of subepithelial deposition of collagen in the colon of collagenous colitis and in the small intestine of coeliac disease, it has been suggested that the colonic collagen band might reflect a toxic effect of gluten on colonic epithelium. However, thickening of the colonic collagen band during strict gluten free-diet, as observed in one of our cases, is not in favour of this hypothesis.
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We report three examples of toxic acute colitis which occurred after ingestion of colocynth (Citrullus colocynthis) for ritual purposes. The prominent clinical feature was dysenteric diarrhoea; colonoscopic changes included congestion and hyperaemia of the mucosa with abundant exudates but no ulceration or pseudopolyp formation. A causal relationship between colonic injury and the intake of colocynth was supported by the following features: (1) the pharmacology of the colocynth extract ingested; (2) the temporal relationship between colocynth intake and clinical onset (eight to 12 h); (3) the rapid recovery within three to six days, with normal endoscopy at day 14; (4) the absence of other possible causes for the observed patterns, except in one case, in which a concomitant intestinal infection with Clostridium perfringens Type A was discovered; (5) the specific pathological features. Colonic biopsies taken 27, 44, and 72 h after colocynth intake showed: erosions with fibrino-purulent exudate, early fibrosis of the lamina propria, hyaline thickening of the superficial epithelial basal membrane. These pathological features completely disappeared within 14 days in all three cases.
Malignant lymphomas occurring in patients with AIDS are usually derived from the B-cell lineage while T-cell malignant lymphomas are very rare in these patients. We report a HIV seropositive 29-year-old homosexual man in whom cervical lymph node biopsy showed an atypical lymphoproliferative process. On morphological and paraffin section immunohistochemical grounds the possibility of Hodgkin's disease (HD) mixed cellularity was initially suggested, but frozen section immunohistochemical studies revealed that the cellular infiltrate exhibited an aberrant pan T immunophenotype and consequently the diagnosis of peripheral T-malignant lymphomas (T-ML) was made. However, genotypic studies would be required to definitely confirm this diagnosis, in such cases. In our case, varying numbers of small and medium-sized cells were positive for both Leu 3/CD4 and Leu 2/CD8 whereas some large cells reacted only with Leu 3/CD4 antibody. Some medium-sized, large and giant cells showed cytoplasmic positivity for Leu M1/CD15. Furthermore, the positivity of many large and giant cells with the activation markers BerH2/CD30, Ki-1/CD30, Tac/CD25 and HLA-DR suggested an activation state for these cells. Our findings emphasize the usefulness of frozen section immunohistochemical methods in order to investigate the spectrum of lymphoid malignancies occurring in HIV seropositive patients, and confirm results of previous studies which stressed the diagnostic difficulties that may appear in distinguishing HD from peripheral T-ML.
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The authors report a case of a primary melanoma of the esophagus, which is a rare neoplasm with poor prognosis. The diagnosis of malignant melanoma was suggested, on endoscopic biopsies, because of the presence of large malignant cells with abundant cytoplasm containing melanin pigment positive in the Fontana-Masson's reaction. These cells were immunoreactive for S100 protein while they did not show staining for antiepithelial membrane antigen and anti-keratin antibodies. Moreover ultrastructural study of the tumor cells revealed cytoplasmic aberrant premelanosomas. An important histological feature of the present case, which confirmed the primary oesophageal nature of the tumor was the presence of foci of melanosis (benign melanocytes) within the oesophageal epithelium which was adjacent to the melanoma. The variation in the histology of malignant melanoma may provide difficulties in diagnosis especially if one is limited to study a small biopsy specimen. In such cases other diagnoses including epidermoid carcinoma with prominent spindle-cell features and sarcomas should be eliminated by means of immunohistochemical and ultrastructural studies. The particular feature of our case, which apparently has not been reported previously, is the identification of the melanosis two years prior to the diagnosis of the malignant melanoma. The present study indicates that the identification of this lesion is of important diagnostic interest and might lead to the follow-up surveillance of the melanosis by multiple biopsies.
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Primary gastrointestinal T-cell malignant lymphomas (T-ML) are very rare. In this case report we describe a primary gastric tumor with local lymph node involvement. On the basis of histologic, immunohistochemical, and electron microscopic studies, the authors classified this tumor as a pleomorphic T-ML, large cell variant with peripheral helper/inducer T-cell phenotype (Leu1/CD5+, Leu4/CD3+, Leu5/CD2+, Leu9/CD7+, and Leu3/CD4+). The extreme pleomorphism of lymphoma cells, the numerous giant cells, and the presence of tumor nodules with two or three concentric layers were the three striking morphologic features of our case. Tumor cells showed an inconstant but true positive staining with anti-LeuM1/CD15 and LeuM3/CD14 antibodies. Vimentin positivity could be related to the presence of intermediate filaments at ultrastructural level. Neuron-specific enolase reactivity was a peculiar but unexplained feature. Furthermore, the positivity of the surface markers Ki-1/CD30, anti-Tac/CD25 and HLA-DR, and the nuclear marker Ki-67 suggested an activation state and a high proliferative activity of the tumor cells. This study emphasizes the usefulness of combined pathologic methods in order to rule other diagnoses such as undifferentiated carcinoma, malignant melanoma, malignant histiocytosis, B-cell lymphoma and interdigitating reticulum cells sarcoma, in view of an extremely polymorph tumor proliferation. This is apparently the first completely documented case report of a primary gastric pleomorphic T-ML of peripheral T-cell origin.
The morphologic, biochemical, and mechanical abnormalities of connective tissue fibrous proteins in Marfan's syndrome have been well studied, and their role in cardiovascular complications is well accepted. Less is known, however, about the state of the amorphous components of the aortic connective tissue. In the course of a study of transmural transport in blood vessels, we have had the opportunity to study dystrophic aorta from two young men who survived elective surgery; both with aortic insufficiency (AI) histologically compatible with Marfan's syndrome. One had recurrent chronic dissecting aneurysm (RCDA) as well. The aorta of the first (but not the second) was histologically compatible with Marfan's syndrome. Fresh specimens of intact ascending aorta were incubated in Krebs solution, pH 7.4, containing 125I-labelled bovine serum albumin for 2 h at 37 degrees C. The samples were then frozen, and serially sectioned in the plane of the lumenal surface. The radioactivity of the 20-micron thick sections was then determined, and expressed as a tissue/labelled solution concentration ratio. Transmural profiles of these ratios revealed no difference between the aorta of the RCDA patient with non-specific aortic dystrophy, and that of a 70-year-old man undergoing aortocoronary bypass. However, in the patient with aortic histology compatible with Marfan's syndrome, the average media concentration ratio was 5-fold less (4% vs. 20%).
We present the fourth case report of an histiocytoid hemangioma of the heart, which was found incidentally, as in the other three reported cases, in a 50 year old man during surgery for congenital heart disease. A polypoid sessile reddish tumor with a smooth surface, which measured 2 cm in diameter, was found in the right atrium. No necrosis nor cystic areas were seen. Histologic evaluation revealed a proliferation of histiocytoid or epithelioid cells, whose arrangement varied a great deal within the infiltrate. Sometimes, the cells were isolated but in most places they were arranged in sheets or nests surrounded by connective tissue. Some of these nests were compact whereas in others a lumen formation, frequently occupied by erythrocytes, could be appreciated. A striking feature was the presence of vacuolated tumor cells sometimes assuming a signet-ring configuration. Some large vascular spaces were lined by histiocytoid or epithelioid endothelial cells, many of which protruded into the lumen. Multinucleated tumor cells were occasionally observed. Mitoses were rare (up to 1/10 HPF) and scattered inflammatory cells such as lymphocytes, macrophages, neutrophils and eosinophils were seen. Histologic appearance of our patient's tumor is identical to the histiocytoid hemangioma of Rosai et al., which is an ubiquitous endothelial benign neoplasm.
We report an aggressive primary T-immunoblastic lymphoma of the small intestine without blood involvement or associated celiac disease. Grossly, the tumor was composed of multiple disseminated ulcerated, infiltrating, or protuberant nodular lesions. Immunologic investigation showed that lymphoma cells were of peripheral (post-thymic) T-cell origin and expressed the phenotype associated with cytotoxic-suppressor subset (Leu4/CD3+, Leu9/CD7+, Leu2/CD8+, Leu11/CD16+, Leu 7/NKcells+, FcIgG+, HLA-DR+, anti-Tac/CD25+, Ki-1/CD30-, Leu1/CD5-, Leu5/CD2-, Leu3/CD4-). A particular morphologic feature of this case is the presence of numerous azurophilic granules within the lymphoma cells, identified as lysosomes by cytochemical and ultrastructural studies. In view of recent immunologic evidence that normal cytotoxic/suppressor T-cells selectively reside within the epithelium of the normal bowel and some of them contain azurophilic granules, it could be suggested that our patient's lymphoma represents a malignant counterpart of these lymphocytes. Furthermore, the aggressive character of this T malignant lymphoma (T-ML) could be related to the expression of T-cell activation markers HLA-DR and Tac/CD25 and the proliferation-associated antigen Ki-67 on a high proportion of tumor cells.