Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P06126 (CD1a)
 2,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis characterized by xanthomatous tissue infiltration with foamy histiocytes. It is still controversial whether these histiocytic proliferations represent monoclonal neoplastic populations or are part of a polyclonal reactive process. This is a case report of ECD in a 76-year-old Chinese woman. We investigated the clinicopathological features and clonality of the histiocytes using laser microdissection and a clonality assay based on X-chromosomal inactivation mosaicism in female somatic tissues, as well as on the polymorphism of phosphoglycerate kinase (PGK) and androgen receptor (AR). According to our results, the lesion was composed of lipid-laden histiocytes and focal fibrous tissues. The lipid-laden histiocytes were positive for CD68 and CD163, but negative for CD1a and S-100. Electron-microscopic examination showed no Birbeck granules, but the presence of lipid vacuoles. Moreover, the result of the clonality assay demonstrated that these cells formed a polyclonal population. In conclusion, ECD is a rare non-Langerhans' cell histiocytosis. Its nature may be a non-neoplastic lesion; however, additional studies with larger sample sizes are necessary to conclusively prove our hypothesis.
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PMID:Clonal status and clinicopathological feature of Erdheim-Chester disease. 1933 22

Some studies have demonstrated that Langerhans cell histiocytosis (LCH) is a neoplastic hyperplasia of Langerhans cells, however some researchers consider that clonality should be assessed in more patients with LCH, both at disease presentation and during the disease course. Monoclonality is a major characteristic of most tumours, whereas normal tissue and reactive hyperplasia are polyclonal. To elucidate the nature of Langerhans cells further, the present study investigated the clinicopathological features and clonality of three cases of LCH in female patients using laser microdissection and a clonality assay, based on X-chromosomal inactivation mosaicism in somatic tissues and polymorphism of the androgen receptor gene. The results indicated that LCH was composed of Langerhans cells with a characteristic morphological appearance, eosinophils, giant cells, neutrophils and foamy cells. Immunohistochemically, the Langerhans cells were positive for CD1a, S-100 protein and vimentin. The clonality assay demonstrated that the Langerhans cells formed a monoclonal population, showing that LCH is neoplastic. We conclude that LCH is characterized by clonal proliferation, although additional studies with larger sample sizes are required to prove this conclusively.
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PMID:Clonal status and clinicopathological features of Langerhans cell histiocytosis. 2081 48

It is sometimes difficult to differentiate between type B3 thymoma from thymic carcinoma histologically. Given the rarity of these tumours, studies have been limited. A series of 66 thymic neoplasms were reviewed and classified according to the World Health Organization (WHO) scheme. We performed a tissue microarray analysis of surgically resected thymic tumour specimens including 12 thymic carcinomas, 17 type B3 thymomas and 37 thymomas of other types. Percentage and staining intensity of immunohistochemical markers were recorded. Tumour eosinophilia was recorded positive if at least one eosinophilic cell identified. Positive staining of the following markers significantly differentiated type B3 thymoma from thymic carcinoma: cytokeratin 5/6 (15 vs. 3), Mesothelin (0 vs. 5), cytoplasmic androgen receptor (10 vs. 0), CD57 (9 vs. 0), CD5 (0 vs. 7), TdT (lymphocytic) (14 vs. 1), CD1a (lymphocytic) (14 vs. 2), CD117 (1 vs. 9), MOC31 (2 vs. 6), p21 (2 vs. 8), cytoplasmic Survivin (0 vs. 4), and tumour eosinophilia (1 vs. 11). Combining two or three markers was able to differentiate these two tumours with area under the curve percentage of at least 92%. Tumour eosinophilia combined with a panel of immunohistochemistry could differentiate type B3 thymoma from thymic carcinoma.
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PMID:Tumour eosinophilia combined with an immunohistochemistry panel is useful in the differentiation of type B3 thymoma from thymic carcinoma. 2104 86