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)

Sinus histiocytosis with massive lymphadenopathy is an uncommon disease affecting individuals of either sex and of all ages and ethnic backgrounds. We present a case of sinus histiocytosis confined to the skin in a 65-year-old woman whose diagnosis was coincident with herpes simplex virus infection and polybacterial urinary infection, both asymptomatic. The polymerase chain reaction for herpes simplex virus and human herpesvirus-6 from involved skin was negative. We review previously published cases of sinus histiocytosis limited to the skin.
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PMID:Sinus histiocytosis confined to the skin. Case report and review of the literature. 860 Aug 4

Soft tissue Rosai-Dorfman disease (STRDD) is rare, previously reported only as single cases and few series. Simian virus 40 (SV40), a polyomavirus, has been identified in lymphoid processes and has a controversial role in neoplasia etiology. Occasional cytoplasmic pink granular inclusions and nuclear changes led us to explore a viral etiology. Only unpublished STRDD from our files with adequate material, soft tissue location, and diagnostic confirmation were included. Immunohistochemistry and follow-up were obtained. Eighteen STRDD patients, 4 male and 14 female, had 29 lesions; 5 with 2 or more lesions. Ages ranged from 8 to 81 years (mean 42.6 years and median 42.5 years). Soft tissue Rosai-Dorfman disease locations include trunk or proximal extremity (n = 19), distal extremity (n = 5), "abdominal" (n = 3), face (n = 1), and unknown subcutaneous site (n = 1). Sizes ranged from 0.5 to 13.7 cm (median, 2.4 cm). Previous disease included lymphoma, buttocks injection site, diabetes and hypothyroidism, and radiation for chronic dermopathy. No patients had a preceding or concurrent known viral infection; none had lymphadenopathy at present. None were known to be immunocompromised. Soft tissue Rosai-Dorfman disease was rapidly progressing. Initial pathologic diagnosis ranged from Rosai-Dorfman disease or inflammatory pseudotumor to inflammatory malignant fibrous histiocytoma. Grossly STRDDs were multilobulated, tan-yellow, and firm; morphologically, circumscribed, and subcutaneous-based. All had sheets of polygonal histiocytes with abundant pale eosinophilic cytoplasm, emperipolesis, plasma cells, and lymphocytes scattered and within clusters. Focal spindle cell change and mild pleomorphism were each observed in 3 patients; 2 had focal necrosis, none with mitoses. Small granular pink cytoplasmic inclusions and nuclear viral-like changes were observed. By immunohistochemistry, all STRDDs were positive for S100 protein, negative for CD1a, Epstein-Barr virus, and latent membrane protein, yet 3 (all abdominal, 1 multicentric) of the 9 studied were focally positive for cytoplasmic and nuclear SV40 polyomavirus. All were treated by local excision. Follow-up on 14 patients older than 8 to 16 years revealed recurrence in 3 patients with persistent multiple lesions, one with abdominal location. There were no metastases or death from disease. Soft tissue Rosai-Dorfman disease is a rapidly evolving, mostly solitary and nonrecurrent trunk and proximal extremity subcutaneous lesion in middle-aged females. More than one third can have persistent multicentric disease. It is important to recognize STRDD, to separate it from malignancy. Epstein-Barr virus/latent membrane protein was negative but polyomavirus was positive in 3 patients with abdominal STRDD, one with multicentric persistent disease. The relationship of polyomavirus to the evolution of abdominal STRDD should be further explored.
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PMID:Soft tissue Rosai-Dorfman disease: 29 new lesions in 18 patients, with detection of polyomavirus antigen in 3 abdominal cases. 2085 Jun 91

The paper describes a case of diffuse large B-cell lymphoma detected in a patient 13 months after sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) being diagnosed together with active hepatitis B virus infection. Analysis of their observations of patients with sinus histiocytosis and the data available in the literature allowed the authors to identify a few tens of cases with SHML associated with lymphomas. This case and previously described ones in the literature on the association of RDD and lymphomas may suggest with a high degree of probability that patients diagnosed as having RDD in evident lymphadenopathy and the non-typical clinical course of RDD may develop blood cancer.
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PMID:[A case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with diffuse large B-cell lymphoma and chronic hepatitis B virus infection]. 2325 52

Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, has been described involving both lymph nodes and extranodal sites, but extranodal RDD rarely involves the gastrointestinal tract. Although the etiology is unclear, several risk factors have been shown to be highly associated with this disease process, including viral infection and immune alterations. In this article, we present a case of a 79-year-old male with a history of autoimmune atrophic gastritis and multiple carcinoid tumors of the stomach presenting with a new stomach mass. An additional large sigmoid colon mass and adjacent enlarged lymph node was identified through imaging, prior to surgery. Through extensive pathologic analysis, we identified the first case of predominant extranodal RDD involving gastric mucosa and submucosa in a background of atrophic gastritis, with additional involvement of the sigmoid colon. Based on this case and literature review, we further discuss possible risk factors and pathogenesis of this disease process.
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PMID:Extranodal Rosai-Dorfman Disease With Mucosal Involvement of the Stomach in a Background of Autoimmune Atrophic Gastritis. 2972 12