Nonamyloidotic fibrillar glomerulopathy and rheumatoid arthritis.
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Biomedical subjects
Publications and source records attributed to J Forteza.
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There is no definite morphological distinction between phaeochromocytomas and paragangliomas. We, therefore, attempted to determine the universality and differential utility of a panel of tumour markers for diagnosis in formalin-fixed, paraffin-embedded specimens. Antibodies to neuron-specific enolase (NSE), chromogranin, synaptophysin, Leu-7, neurofilaments, cytokeratins, carcinoembryonic antigen (CEA), melanoma antigen HMB-45, S-100 protein and glial fibrillary acid protein (GFAP), were used on 11 phaeochromocytomas and 8 paragangliomas. NSE reactivity was detected in 10 phaeochromocytomas and in all paragangliomas. Chromogranin reactivity was found in all but two cases (one phaeochromocytoma and one paraganglioma). Synaptophysin reactivity was present in 10 phaeochromocytomas and in the 8 paragangliomas. Ten phaeochromocytomas stained for Leu-7, but none of the paragangliomas did. S-100-positive cells (sustentacular or type II cells) were found in 8 phaeochromocytomas and 7 paragangliomas. GFAP stained sustentacular cells of only one paraganglioma. Only in 5 phaeochromocytomas was there a focal reaction by neurofilaments. Cytokeratins, CEA and HMB-45 were never detected. We conclude that NSE, chromogranin, synaptophysin and S-100 protein are useful markers of both types of tumour, whereas GFAP staining is limited to a small number of these neoplasms. Leu-7 reactivity seems to favour diagnosis of phaeochromocytoma rather than paraganglioma, but further studies with larger series are needed to confirm this. Unlike previous reports, we did not find cytokeratin or HMB-45 immunostaining in any case.
Prothymosin alpha (ProT alpha) distribution in human and rat lymph nodes and human tonsils was studied by means of immunohistochemical methods, using specific antibodies raised against thymosin alpha 1. We observed ProT alpha immunoreactivity in lymphoid cells of the germinal centers both in humans and rats. In human tonsils, positive cells were also seen in the basal layer of the surface epithelium. These results support the hypothesis that ProT alpha expression is restricted to actively proliferating cells. Immunoelectron microscopy revealed that ProT alpha was located in the nucleus, mainly in the border between euchromatin and heterochromatin.
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Sixty three cases of Hodgkin's disease are studied (two with lymphonodular predominance, 15 with diffuse lymphocyte predominance, 26 nodular sclerosis, 15 mixed cell and 5 lymphocyte depletion) with a panel of 8 monoclonal antibodies, material routinely used and included in paraffin: Ber H2 (CD30), Leu M1 (CD15), Common Leukocyte Antigen (CD45), L26 (CD20), MB2, UCHL1 (CD45 RO), MTI (CD43) and Epithelial Membrane Antigen. Ber H2 turned out to be the most usefull marker, positive in 100% of cases, independently of the histologic type. Positiveness with Leu M1 ranged from 100% (2/2 cases) of lymphonodular predominance, to 53.3% (8/15 cases) of diffuse lymphocyte predominance. The reactivity of the rest was variable, 1 though it is note worthy the positiveness of B markers (L26, MB2) in the two cases with lymphonodular predominance. In the other subtypes, reactivity with L26 was greater than that with MB2. T cell marker expression was minimal, except for the positiveness in 40% of cases (2/5) of the lymphocyte depletion type. In addition, the results of other series are revised and as a result the possible histogenesis of Hodgkin's disease is discussed.
Two cases of Alagille syndrome are reported, father and son. This, a 6-year-old boy, presented with neonatal cholestasis but thereafter evolved with progressively decreasing jaundice and persisting pruritus. A liver biopsy confirmed the absence of intrahepatic bile ducts with preservation of hepatic architecture and no fibrosis. The patient had a characteristic phenotype: short stature, triangular face, deep eyes with hypertelorism, partial embryotoxon and data of peripheral pulmonary artery stenosis. His father died 43-year-old by a hepatocarcinoma. His liver biopsy showed also absence of intrahepatic bile ducts. In addition to the association Alagille's syndrome hepatocarcinoma (previously reported in six cases, three into the same family), it should be stressed in this case the long survival and the fact he had many children: the case with Alagille's syndrome, five children in good health, and one who died shortly after birth.
General opinion is that retinoblastomas, though not everyone agrees with that view. Some authors suggest that retinoblastomas are derived from a primitive retinal cell able to differentiate into both neuronal and glial cell lines. The aim of the present work was to study immunohistochemically the expression of neuronal and astrocytic markers in retinoblastomas and at the same time the presence of the oncofoetal antigens carcinoembryonic antigen (CEA) and alpha Foeto Protein (AFP), since patients with retinoblastomas often show high oncofoetal antigen in serum levels. For this purpose we employed the streptavidin-biotin immunoperoxidase technique in 13 cases of retinoblastoma to evaluate the presence and distribution of neuron-specific enolase (NSE), neurofilament protein (NF), glial fibrillary acidic protein (GFAP), S-100 protein, CEA and AFP. All 13 tumours studied stained for NSE. Seven of them showed GFAP- and S-100 positive perivascular glial cells as well as cells distributed randomly in the tumour that were interpreted as non tumour cells. All 13 retinoblastomas lacked detectable NF, CEA, and AFP. These results support the idea that retinoblastomas are neuronal tumours, although retinal glial cells may become incorporated in the tumour and proliferate in response to the tumour.
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Synaptophysin is a membrane glycoprotein (Mr 38,000) found in small vesicles with clear content. It is present in neurons and in a variety of neuroendocrine cells and neoplasms, including the neuroendocrine carcinoma of the skin (Merkel cell tumor). We report the presence of synaptophysinlike immunoreactivity in normal Merkel cells of the pig-snout epidermis and vibrissae. The immunostaining was primarily localized in the Merkel cell side opposite to the nerve terminal. This fact suggests that in Merkel cells, as well as in other neuroendocrine cells, synaptophysin could occur in small clear vesicles (accumulated in Merkel cells in the side opposite to the neurite) and not in the secretory granules themselves (which are concentrated in the cellular side apposed to the neurite).
Liver involvement in non-Hodgkin's lymphoma is relatively frequent, being found in approximately half of the post-mortem studies. However, the clinical presentation of lymphoma mimicking primary liver disease is uncommon. A case of non-Hodgkin's lymphoma (the recently described T-cell-rich B-cell lymphoma variant) is presented, where the initial and predominant manifestations were of hepatic origin, resembling non-neoplastic acute liver disease.
A 60 year-old white woman presented with sudden painless dysphagia, hoarseness and dysphonia. A diagnosis of sarcoidosis was made based on bilateral hilar lymphadenopathy, gallium uptake, elevated serum angiotensin-converting enzyme levels, as well as non-caseating granulomatous lymphadenitis in a prescalenic node. Symptoms were attributed to isolated vagus neuropathy, a rare form of presentation of neurosarcoidosis.
A 30-year-old male with a past history of nodular lymphocyte predominance Hodgkin's disease in apparent complete remission for two years received a liver transplantation because of fulminant liver failure. Histopathological examination of the explanted liver showed massive infiltration by Hodgkin's disease. In spite of a nodal recurrence of Hodgkin's disease, the patient is alive and in excellent general condition six years after liver transplantation.