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

The immunophenotype and proliferation fraction have been investigated in 26 cases of Langerhans' cell histiocytosis (LCH). In all cases LCH cells were positive for S-100 protein, CD1a, or both. In most cases LCH cells expressed the macrophage-associated marker CD68 and in two cases they contained lysozyme. Expression of both cytoplasmic CD2 and CD3 was observed in cryostat sections. An unexpected finding was the presence of placental alkaline phosphatase in LCH cells. Langerhans' cells in normal skin were negative for both CD2 and CD3, but a proportion contained placental alkaline phosphatase. In four cases of Rosai-Dorfman disease the histiocytic cells, which share certain immunophenotypic properties with Langerhans' cells, also were positive for placental alkaline phosphatase. A significant proportion of LCH cells stained positively with the antibody to proliferating cell nuclear antigen and also with the proliferation marker Ki-S1. A good correlation between the percentage of Ki-67-positive and proliferating cell nuclear antigen- and Ki-S1-positive cells, respectively, was observed. Thus, in comparison with their putative precursors, LCH cells have an aberrant phenotype and are proliferating locally. This might suggest that LCH is a neoplastic rather than a reactive process.
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PMID:Langerhans' cell histiocytosis (histiocytosis X): immunophenotype and growth fraction. 769 Jul 35

Rosai-Dorfman disease is a rare, idiopathic, benign, and self-limiting histiocytic proliferative disorder. A 26-year-old man presented with a single massive cutaneous nodule (reaching 30 cm in diameter) on the left shoulder and back for 15 months. The routine hematological and biochemical tests were normal. Magnetic resonance scanning showed the lesion involved the skin, subcutaneous tissue, and subjacent muscle group, accompanied by obvious lymph node enlargement in the left part of the neck, supraclavicular fossa, and axillary fossa. The histopathology of the left cervical lymph node revealed diffuse effacement of the normal nodal architecture, with patchy chronic inflammatory cell infiltrates comprising lymphocytes and sheets of histiocytes. Some histiocytes contained lymphocytes within their pale cytoplasm. Many multinucleated giant cells were found; however, caseating granulomas were not seen. The skin and muscle biopsy specimen obtained from the back revealed infiltrating lymphocytes and histiocytes diffusely distributed in the dermis, subcutaneous tissue, and crevices of the muscle fibers. The phenomenon of emperipolesis and the presence of multinucleated giant cells were also seen. Immunohistochemical staining revealed that the histiocytes were positive for S-100 protein and CD68 but negative for CD1a. Immunophenotyping of the infiltrating lymphocytes indicated positive reactions to CD3, CD45RO, CD5, CD7, CD4, CD8 (partly), CD79a, CD20 (partly), and Ki-67 (<1%). The final diagnosis was Rosai-Dorfman disease. Owing to the extensive and deep involvement of the subcutaneous tissue and muscles, the patient did not undergo surgery to excise the massive skin nodule. The lesion showed no obvious change at the 12-month follow-up.
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PMID:Rosai-dorfman disease with massive cutaneous nodule on the shoulder and back. 2567 35

A lymphoplasmacyte-rich meningioma (LPRM) is an extremely rare variant of meningioma. Here, we report a case of atypical LPRM with increased mitosis in a 55-year-old man. Preoperative magnetic resonance imaging suggested meningioma with brain invasion in the left tentorium cerebelli. Histological examination revealed sclerotic fibrosis and dense lymphoplasmacytic infiltration; based on these findings, the differential diagnosis included LPRM and fibroinflammatory lesions of the dura, such as idiopathic hypertrophic pachymeningitis (IHP), IgG4-related disease (IgG4-RD), and Rosai-Dorfman disease. Epithelial membrane antigen (EMA) immunostaining highlighted sheets of meningothelial cells, which strongly supported the diagnosis of meningioma. Although brain invasion was suspected in radiologic image, no histologic evidence of brain invasion was found. However, the mitoses were observed to be 8/10 high power fields, along with increased Ki-67 labeling index with focal spontaneous necrosis, and the final pathologic diagnosis was atypical LPRM. IgG4-RD was ruled out, because IgG4 counts and the IgG4:IgG ratio of plasma cells did not meet the diagnostic criteria for IgG4-RD. To date, only one case of LPRM with brain invasion has been reported as atypical LPRM. This case is therefore the second case of atypical LPRM with increased mitosis that histologically mimicked IHP.
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PMID:Report of a rare case of atypical lymphoplasmacyte-rich meningioma in the tentorium mimicking idiopathic hypertrophic pachymeningitis. 2690 1