Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The aim of this study was to investigate the regional importance of gamma delta T cells in cutaneous lesions of Langerhans' cell histiocytosis. Six cases of Langerhans' cell histiocytosis were investigated by immunohistochemical techniques (alkaline phosphatase-antialkaline phosphatase complex and indirect immunoperoxidase). Increase of gamma delta T cells was observed in 3 cases of Langerhans' cell histiocytosis. In these cases up to 30% of CD3+ cells stained with an anti-TCR gamma delta monoclonal antibodies and in two of them gamma delta T cells showed a marked epidermotropism. In the specimens of the remaining three cases gamma delta T cells were found in an overall percentage of 5% of CD3+ cells, but in two cases a significant increase of epidermal gamma delta T cells was observed. The finding of numerous gamma delta T cells in Langerhans' cell histiocytosis is provocative and supports the suggestion of a functional relationship between gamma delta T cells and LCH cells.
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PMID:T-Lymphocytes bearing the gamma delta T-cell receptor in cutaneous lesions of Langerhans' cell histiocytosis. 849 49

We describe the clinicopathologic features of 15 patients who had histiocytic lesions that followed acute lymphoblastic leukemia (ALL). Twenty-one separate histiocytic lesions were evaluated that covered a wide spectrum, some conforming to the usual categories of juvenile xanthogranulomas (5), Langerhans' cell histiocytosis (1), Langerhans' cell sarcoma (4), Rosai-Dorfman disease (1), and histiocytic sarcoma (4). Most were atypical for the category by histology, phenotype, or abnormally high turnover rate. Seven low-grade lesions defied easy categorization and were characterized only as "atypical histiocytic lesion" following ALL. For those evaluated, the molecular signature of the prior leukemia was present in the histiocytic lesion. In 3 of 15 patients, the leukemia and histiocytic lesion shared immunoglobulin H or monoclonal TCR gene rearrangements and, in 4 of 15 patients, clonal identity was documented by fluorescence in situ hybridization. Four patients died of progressive disease, 3 of whom had histiocytic sarcoma and 1 who had an atypical lesion. One patient died of recurrent ALL. The other 10 patients are alive, 7 after recurrences and treatment with surgery and/or chemotherapy. The post-ALL lesions are more aggressive than their native counterparts, but despite the demonstration of the presence of the leukemia signature in 7 of 15 patients, the prognosis is generally favorable, except for patients with histiocytic sarcoma. It remains unclear whether the histiocytic lesions arise as a line from the original ALL or whether transdifferentiation is involved.
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PMID:Clinicopathologic features of histiocytic lesions following ALL, with a review of the literature. 1964 34