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 unusual disease characterized by prominent lymph node enlargement, especially in the cervical region. The clinical course is benign although prolonged, and no specific treatment is required. The etiology and pathogenesis are unknown. Four cases of sinus histiocytosis are reported, one in association with mediastinal lymph node enlargement and two in patients with orbital enlargement. One other patient had cervical masses. The radiologic features are reviewed to increase awareness of this entity as a cause of lymph node and orbital enlargement.
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PMID:Sinus histiocytosis: some radiologic observations. 10 46

A comparative study was made on different morphological pictures of the sinuses of the regional lymph nodes in gastric cancer patients with T1--4N0M0 with a 5-year survival, also the influence of age, depth of invasion, size and histological structure of the tumor on sinus histiocytosis was studied. Marked sinus histiocytosis, contrary to sinus fibrosis, was found to be a favourable prognostic sign. Sinus histiocytosis is influenced in a somewhat directly proportional way by the tumor size and histological differentiation. The given material rendered impossible to reveal any effect of age and depth of tumor invasion on sinus histiocytosis.
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PMID:[Prognostic significance of sinus histocytosis of the regional lymph nodes in stomach cancer]. 46 74

Sinus histiocytosis with massive lymphadenopathy (SHML) was found in two siblings, both of whom had retropharyngeal involvement. In one of the children the retropharyngeal swelling appeared to produce obstructive symptoms, requiring excision of the involved lymph nodes. These cases appear to constitute not only the first proven familial occurrence of SHML but also the only reported instances of retropharyngeal involvement. SHML is an uncommon disease of unknown etiology that must be distinguished from other diseases associated with marked lymphadenopathy, particularly as its course is generally benign and not altered by any known form of medical treatment.
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PMID:Sinus histiocytosis with massive lymphadenopathy. Report of its occurrence in two siblings with retropharyngeal involvement in both. 65 69

Three hundred and two carcinomas of the female breast were studied histologically with special reference to the morphologic aspects of the tumor, its surrounding host tissue and the regional lymph nodes. The nuclear grade of the tumor was positively correlated with the five year survival rate of the patient. Tumor metastases in the regional lymph nodes were observed to be a sign of a poor prognosis. The stromal lymphocyte and mast cell reactions did not correlate with the frequency of nodal metastases or the five year survival rate. Sinus histiocytosis in the lymph nodes was a sign of favorable five year survival because of its presence in cancer-free nodes only. The paracortical activity of the lymph nodes was an important determinant of whether or not tumor metastases appear in the node.
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PMID:Tumor-host interrelationships in carcinoma of the female breast. 66 8

One hundred and thirty eight gastric carcinomas were studied histologically with special reference to the morphology of the tumor, its surrounding tissues and the regional lymph nodes. A special search was focused on the morphologic manifestations of possible host factors in association with gastric carcinoma. The most prominent findings were as follows: 1. The nuclear grade of the tumor was positively correlated with the 5-year survival rate of the patients. 2. The content of tumor-derived mucus was not a prognostic determinant. 3. The intensity of the stromal lymphocyte and plasma cell reactions did not affect the prognosis but was inversely related to the frequency of nodal metastases. 4. Sinus histiocytosis and nodal mast cell reactions were an important determinant of whether nodal metastases appear or not. 5. An active paracortical area of the lymph node was almost incompatible with the appearance of nodal metastases.
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PMID:Prognostic factors in gastric carcinoma. 68 79

Sinus histiocytosis with massive lymphadenopathy (SHML) is a newly recognized, distinct, pseudolymphomatous benign entity with very characteristic microscopic features. Most patients are children or young adults with massive painless cervical adenopathy, although other node groups and extranodal sites often are involved. Sixteen patients with SHML involving the upper respiratory tract and/ or salivary gland are presented. Nine of the patients had ear, nose, and throat (ENT) manifestations at the time of presentation. In most cases the ENT involvement resulted in prominent clinical symptoms. Treatment included surgery, antibiotics, irradiation, chemotherapy, and steroids, frequently in combination, but no consistent pattern of response emerged from the study. These 16 patients were very similar clinically to patients with SHML who did not have ENT disease, indicating that extranodal involvement is not associated with more aggressive disease.
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PMID:Sinus histiocytosis with massive lymphadenopathy. 71 24

Sinus histiocytosis is a newly recognized benign disease affecting mainly children and young adults and usually having a protracted clinical course that is relatively unaffected by therapy. This paper describes four additional patients who had orbital involvement initially and reviews the salient clinical and histopathologic features of this entity. The outstanding clinical feature is cervical lymphadenopathy. Associated findings include low-grade fever, anemia, leukocytosis, and elevated IgG levels. A small percentage of patients develop proptosis with palpable orbital tumors. Such patients may not have appreciable lymphadenopathy. Progressive proptosis may lead to exposure keratitis, corneal ulceration, endophthalmitis, and loss of the eye. Histopathologically, the lymph nodes and orbital mass show a proliferation of large histiocytes intermixed with a variable proportion of lymphocytes and plasma cells. Lymphocytes and occasionally other cells derived from the hematopoietic system are commonly seen within the cytoplasm of the histiocytes.
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PMID:Orbital involvement in 'sinus' histiocytosis. A report of four cases. 92 80

Sinus histiocytosis with massive lymphadenopathy is a rare disorder of the reticuloendothelial system with predilection for black children.
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PMID:Widespread bone involvement in sinus histiocytosis. 108 61

A case of sinus histiocytosis with generalized lymphadenopathy--Rosai-Dorfman disease--in a 54 year old male is presented. The diagnosis was made by biopsy of the lymph nodes and histopathological examination of the affected organ. Treatment with prednisone and cyclophosphamide induced clinical improvement. The diseases clinical advanced stage and organ localization and the immunosuppressive therapy resulted in a opportunistic infection--pneumocystidosis and death of the patient within half a year after starting of therapy.
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PMID:[Sinus histiocytosis with generalized lymphadenopathy--Rosai-Dorfman disease]. 129 Sep 82

Sinus histiocytosis with massive lymphadenopathy involving organs other than the lymph nodes is a rare event. A case of SIIML presenting with multiple skin and subcutaneous nodules and multiple osteolytic lesions is described. A search of the Indian literature revealed many cases of nodal SIIML, but none of the reported cases had prominent extranodal involvement.
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PMID:Extranodal sinus histiocytosis with massive lymphadenopathy--a case report. 129 10


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