Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 37-year-old Bangladeshi presented with large bilateral masses involving the hilus of the kidneys. No lymphadenopathy was noted. Nephrectomy was performed. Histopathologically, it revealed a lymphohistiocytic and plasma cell inflammatory tumoral proliferation with characteristic lymphophagocytosis by the S100-positive-CD1-negative histiocytes. Extranodal presentation of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) should be entertained in the differential diagnosis of bilateral masses involving the kidneys.
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PMID:Unusual bilateral renal histiocytosis. Extranodal variant of Rosai-Dorfman disease. 145 86

Five cases of Rosai-Dorfman histiocytosis (RDH) (also called Sinus Histiocytosis with Massive Lymphoadenopathy; SHML) have been studied by immunohistochemical methods with heteroantisera and monoclonal antibodies. One case was also studied by Southern blot hybridization analysis with DNA probes specific for T cell receptor beta chain and immunoglobulin heavy chain. Immunophenotyping of large histiocytes, characteristic of RDH, evidenced the presence of S-100 protein and the absence of CD1 and other markers usually found in histiocytes and macrophages. DNA hybridization study showed the absence of clonal T or B lymphoid populations.
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PMID:Immunohistological analysis of Rosai-Dorfman histiocytosis. A disease of S-100 + CD1-histiocytes. 311 Oct 77

The May 2002 COM. A 38-year-old man presented with new onset seizures and a 69-year-old woman presented with bilateral headaches and episodes of syncope. Both were found to have extra-axial masses that were contrast-enhancing and thought to be meningiomas. Both had complete resection. Microscopic examination revealed an inflammatory lesion composed of plasma cells, scattered lymphocytes and numerous large histocytic cells, which exhibited emperi polesis and were CD1 a negative, but positive for CD68 and S100. The diagnosis of Destombes-Rosai-Dorfman Disease (DRDD) was rendered. Both cases had good long-term outcome. The differential diagnosis of inflammatory masses in the dura (plasmacytoma, lymphomas, plasma cell fibroma, angiofollicular hyperplasia [Castleman's-disease] and Langerhan's cell histiocytosis) are discussed.
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PMID:May 2002: 38-year-old man and 69-year-old woman with dural based masses. 1240 40