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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Rosai-Dorfman syndrome is a benign disease, one of its commonest affected sites being the cervical lymph nodes. This disease should be considered in the differential diagnosis of a cervical lymphadenopathy. The syndrome often begins with a chronic rhinitis, and is accompanied by sinusitis, tonsillitis or pharyngitis; blood tests indicate chronic infection. The pathogenesis of the disease is still unknown but may include infection or an abnormal autoimmune reaction. The diagnosis is established by the findings of characteristic histiocytes with haemophagocytosis. We report a female patient with persistent cervical lymph node swelling resistant to treatment. The cause was proved by histology to be a Rosai-Dorfman syndrome.
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PMID:[Rosai-Dorfman syndrome. On the differential diagnosis of lymph node swelling]. 156 88

Sinus histiocytosis with massive lymphadenopathy is a well recognised, but rare cause of lymphadenopathy in the first decade of life. Three cases presenting with nodal disease are described. The eyelids were involved in one case. The clinical, laboratory, and biopsy findings are discussed and compared with previously reported cases.
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PMID:Sinus histiocytosis with massive lymphadenopathy. 158 Jun 84

Little is known about the nature of the large intrasinusoidal cells exhibiting cytophagocytosis, which are the histologic hallmark of sinus histiocytosis with massive lymphadenopathy (SHML) (Rosai-Dorfman disease). Using a broad panel of monoclonal and polyclonal antibodies, we analyzed the immunophenotype of the cell infiltrates in seven lymph node biopsy specimens from five cases of SHML. The SHML cells constantly expressed the S-100 protein, concanavalin agglutinin and peanut agglutinin lectins, and monocyte-macrophage-associated antigens CD 11c, CD 14, CD 33, CD 68, and LN 5. Labeling with other antimacrophage antibodies was extremely variable, with some (MAC 387, lysozyme) restricted to clusters of SHML cells and others (CD11b, CD 36, alpha-1-antichymotrypsin) staining only scattered cells. The CD 1a antigen was found on some cells in only one case, whereas HLA-DR and the HLA-DR-associated invariant chains were absent. The heterogeneity of SHML cell marker expression might be related to the local content of factors (eg, cytokines) capable of modulating the phenotype of monocytes and derived cells. All cases presented with huge amounts of medium-sized mononuclear cells accumulated in the sinuses and intersinusoidal tissue. These cells expressed the S-100-/CD 11b+/CD 11c+/CD 14+/CD 16+/CD 33+/CD 36+/lysozyme+/MAC 387+/HLA-DR+ phenotype. These recently immigrated monocytes might represent the immediate precursors of SHML cells.
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PMID:Immunophenotypic characterization of the cell infiltrate in five cases of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). 159 87

Sinus histiocytosis with massive lymphadenopathy (SHML) is a newly recognized disorder. The etiology of this disease is unknown. An exaggerated response to an offending agent such as the Epstein-Barr virus or Klebsiella bacteria has been postulated. Its course is usually benign. Cervical adenopathy is seen in 97% of the patients, while 30% of patients have nodal involvement in other sites, and 30% have extranodal involvement. There is a 7% mortality rate that occurs primarily in patients with immunologic defects. Corticosteroids ameliorate the constitutional symptoms, but cyclophosphamide appears to have the most beneficial effect. This article presents the case of a patient with SHML who demonstrated elevated Epstein-Barr virus titers.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with elevated Epstein-Barr virus titers. 166 27

Whereas Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy is well described in lymph nodes and other organs, it is frequently not recognized in soft tissue. We studied the clinical and histologic features of 23 previously unreported soft tissue lesions from 17 patients (13 females, 4 males) who were 24 to 66 years of age (mean, 46 years). These lesions involved the extremities (12, 52%), trunk (6, 26%), head and neck (3, 13%), and the retroperitoneum (2, 9%). Associated lymph node involvement was present in four cases; most patients were asymptomatic. RDD of soft tissue had more subtle histologic features than its lymph node counterpart. Emperipolesis was less conspicuous and proliferating histiocytes were frequently spindled, associated with collagen deposition, and arranged in a vague storiform pattern with scattered lymphoplasmacytic aggregates. These features led to a variety of diagnoses, including benign inflammatory and fibrohistiocytic lesions (13 cases) as well as lymphoma and malignant fibrous histiocytoma (three cases). RDD was correctly diagnosed in only one case. Diagnosis was confirmed in 16 of 18 lesions by detection of S-100 protein and histiocytic markers KP1 (12 of 13) and lysozyme (eight of 11) in the characteristic histiocytes. Recognition that RDD of soft tissue occurs in an older patient population than does nodal RDD and that it mimics fibrous and inflammatory lesions of soft tissue is important.
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PMID:Rosai-Dorfman disease of soft tissue. 173 47

The histiocytoses of childhood include Langerhans' cell histiocytosis, haemophagocytic syndrome (familial and reactive), sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease), juvenile xanthogranuloma and malignant histiocytosis. These disorders show wide variation in their clinical presentation, prognosis and genetic implications. All are characterized by localized or generalized proliferation of histiocytes, but they differ in their morphology, histochemical and immunochemical staining patterns and electronmicroscopical features. On the basis of a comprehensive clinical history and critical interpretation of morphology and immunocytochemistry using an appropriately selected panel of antibodies, a diagnosis can be reached in the majority of cases.
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PMID:The histiocytoses of childhood. 139 47

We report one case of pseudotumoral salivary location of the Rosai-Dorfman syndrome, with a favorable outcome, occurring in a 32-year-old man from Guadeloupe. The clinical, biological and clinicopathological features of this syndrome, as well as the pathogenetic hypotheses, are summed up. In this case, no bacterial, fungal, parasitic or viral agent was discovered. The subject presented with no immune abnormality likely to account for the persistence and intensity of the histiocytic infiltration with lymphophagocytosis associated to the lymphoplasmocytic infiltration. The rarity of ENT, and more specifically salivary, locations is emphasized. The importance of the detection of possible immune abnormalities in such patients is demonstrated by the outcome of the syndrome being more often unfavorable when such abnormalities are present.
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PMID:[Pseudotumoral salivary localization of Destombes-Rosai-Dorfman syndrome. Hemophagocytic histiocytosis. Diagnosed by bilateral submandibular and parotid involvement]. 175 44

A 28 year-old female patient was operated for acute abdominal pain. At the surgical intervention the mesenteric nodes turned out to be enlarged and the appendix was apparently normal. Microscopically, the changes observed were of the Rosai-Dorfman disease type in the appendix as well as in the nodes. A salmonella D was cultivated in the mesenteric nodes. This association, which has not been described in the literature, permits us to comment clinicopathological aspects, poorly defined, with special reference to the possibility that some instances of Rosai-Dorfman disease could be secondary to an infectious process.
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PMID:[Rosai-Dorfman disease of the mesenteric lymph nodes and appendix. Secondary to salmonellosis?]. 180 93

The paper reviews the results of an extensive morphological analysis of 49 lymph nodes draining the area of histologically evidenced tumor on the basis of immunomorphological changes by the quantitative and semiquantitative methods. Reactive changes in different topographic zones being a morphological manifestation of the immune response to the presence of tumor depend on the type and localization of the tumor. Sinus histiocytosis is the most significant morphological reaction in the analyzed draining nodes. The intensity of sinus histiocytosis could, by itself, represent an important factor in a morphological recognition of the lymph node draining the tumor area.
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PMID:[The present approach to the morphologic analysis of lymph nodes draining a tumor area]. 182 42

A prospective study of comparable Japanese and British breast cancer patients treated by radical mastectomy confirmed previously reported findings that sinus histiocytosis and germinal center hyperplasia are more frequently seen in axillary lymph nodes from Japanese than in those from British patients. In Japanese, but not British, cases of either of these two morphologic findings had favorable prognostic significance for recurrence. Sinus histiocytosis also had favorable prognostic significance in Japanese cases for five year survival. In a separate review of axillary nodes from Japanese autopsy cases sinus histiocytosis was absent, suggesting that this finding in Japanese breast cancer cases was related to presence of the disease.
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PMID:Breast cancer in English and Japanese women: prognostic significance of sinus histiocytosis and germinal center hyperplasia in axillary lymph nodes. 187 61


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