| pubmed-article:3925627 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:3925627 | lifeskim:mentions | umls-concept:C0007634 | lld:lifeskim |
| pubmed-article:3925627 | lifeskim:mentions | umls-concept:C0039194 | lld:lifeskim |
| pubmed-article:3925627 | lifeskim:mentions | umls-concept:C0205462 | lld:lifeskim |
| pubmed-article:3925627 | lifeskim:mentions | umls-concept:C1801959 | lld:lifeskim |
| pubmed-article:3925627 | lifeskim:mentions | umls-concept:C0011155 | lld:lifeskim |
| pubmed-article:3925627 | lifeskim:mentions | umls-concept:C0205161 | lld:lifeskim |
| pubmed-article:3925627 | lifeskim:mentions | umls-concept:C1441165 | lld:lifeskim |
| pubmed-article:3925627 | lifeskim:mentions | umls-concept:C1441616 | lld:lifeskim |
| pubmed-article:3925627 | lifeskim:mentions | umls-concept:C0728938 | lld:lifeskim |
| pubmed-article:3925627 | lifeskim:mentions | umls-concept:C0332120 | lld:lifeskim |
| pubmed-article:3925627 | lifeskim:mentions | umls-concept:C1514485 | lld:lifeskim |
| pubmed-article:3925627 | pubmed:issue | 1 | lld:pubmed |
| pubmed-article:3925627 | pubmed:dateCreated | 1985-8-22 | lld:pubmed |
| pubmed-article:3925627 | pubmed:abstractText | A 7 month old female infant was affected by a rapidly fatal familial disease highly reminiscent of Omenn's syndrome. She presented with widespread eczematous lesions, hepatosplenomegaly, superficial lymphadenopathy, peripheral blood lymphocytosis, eosinophilia and hyper-IgE. An axillary lymph node was involved by a marked proliferation of T-3 +/T-10-- lymphocytes admixed with S-100+/T-6+/Leu-3a+/Ia + reticular cells which lacked typical LC granules; cell suspension study revealed that 90%-96% of the lymph node cells were T-11+/T-3+ lymphocytes characterized by low expression of Leu-3a and T-8 antigens and by high expression of Ia antigens (52%). Peripheral blood T lymphocytes exhibited a similar distribution of surface phenotypes. The patient died of interstitial pneumonia and an autopsy was performed. The thymus was markedly atrophic and completely devoid of lymphocytes. The peri-arteriolar lymphoid sheets of the spleen were poorly developed and were mainly composed of T-8+ lymphocytes. The mediastinal nodes were rudimentary and were populated by T-3+/T-10+ lymphocytes with low expression of Leu-3a and T-8 antigens. Our results raise the possibility that Omenn's syndrome is a peculiar primary immunodeficiency in which, despite early thymic involution, some abnormal T lymphocytes still develop in the peripheral lymphoid organs. Antigenic triggering of these cells might result in prominent proliferations of T lymphocytes and Langerhans-like cells which lead to the clinical manifestation of the disease. | lld:pubmed |
| pubmed-article:3925627 | pubmed:language | eng | lld:pubmed |
| pubmed-article:3925627 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:3925627 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:3925627 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
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| pubmed-article:3925627 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:3925627 | pubmed:issn | 0174-7398 | lld:pubmed |
| pubmed-article:3925627 | pubmed:author | pubmed-author:UcciniSS | lld:pubmed |
| pubmed-article:3925627 | pubmed:author | pubmed-author:BusincoLL | lld:pubmed |
| pubmed-article:3925627 | pubmed:author | pubmed-author:TAITE FEF | lld:pubmed |
| pubmed-article:3925627 | pubmed:author | pubmed-author:BarsottiPP | lld:pubmed |
| pubmed-article:3925627 | pubmed:author | pubmed-author:PezzellaFF | lld:pubmed |
| pubmed-article:3925627 | pubmed:author | pubmed-author:BonerA LAL | lld:pubmed |
| pubmed-article:3925627 | pubmed:author | pubmed-author:StoppacciaroA... | lld:pubmed |
| pubmed-article:3925627 | pubmed:author | pubmed-author:MiroloMM | lld:pubmed |
| pubmed-article:3925627 | pubmed:author | pubmed-author:Di FazioAA | lld:pubmed |
| pubmed-article:3925627 | pubmed:author | pubmed-author:CassanoA MAM | lld:pubmed |
| pubmed-article:3925627 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:3925627 | pubmed:volume | 407 | lld:pubmed |
| pubmed-article:3925627 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:3925627 | pubmed:authorsComplete | N | lld:pubmed |
| pubmed-article:3925627 | pubmed:pagination | 69-82 | lld:pubmed |
| pubmed-article:3925627 | pubmed:dateRevised | 2006-11-15 | lld:pubmed |
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| pubmed-article:3925627 | pubmed:meshHeading | pubmed-meshheading:3925627-... | lld:pubmed |
| pubmed-article:3925627 | pubmed:year | 1985 | lld:pubmed |
| pubmed-article:3925627 | pubmed:articleTitle | The Omenn's syndrome: histological, immunohistochemical and ultrastructural evidence for a partial T cell deficiency evolving in an abnormal proliferation of T lymphocytes and S-100 +/T-6 + Langerhans-like cells. | lld:pubmed |
| pubmed-article:3925627 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:3925627 | pubmed:publicationType | Case Reports | lld:pubmed |
| pubmed-article:3925627 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
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| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:3925627 | lld:pubmed |
