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 (SHML) also called as Rosai Dorfman disease is a rare histiocytic proliferative disorder of unknown etiology. Histological features currently define it. Persistent painless lymphadenopathy due to expansion of sinuses infiltrated with benign histiocytes and plasma cells and emperipolesis are the characteristic features of SHML. Our study includes seven cases (5 nodal and 2 extranodal) of SHML over a 5-year period whose slides and blocks were reviewed. IHC was performed on the main lesion, from a panel of S100, CD68, LCA, CD20, CD3, CD30, CD43, bcl2, cytokeratin and epithelial membrane antigen. In our series we have work up available in 7 cases out of which a detailed follow-up is available in 5 patients. Out of these 5 patients, 4 have a stable disease, while one developed histiocytic sarcoma after a gap of four years.
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PMID:Sinus histiocytosis with massive lymphadenopathy--a review of seven cases. 1718 39

Emperipolesis is a physiologic or pathologic phenomenon characterized by the presence of intact viable cells within the cytoplasm of another cell. It has been described in normal tissues and in a variety of inflammatory and neoplastic lesions such as Rosai-Dorfman disease, tumors, hematopoietic disorders and rarely lymphomas. Emperipolesis by mesothelial cells is rare. Few cases of mesothelial emperipolesis of neoplastic lymphocytes in pleural effusions involved by lymphomas have been reported in the literature. Its etiopathogenesis and significance are controversial and speculative. We report a case of a 36-year-old man who presented with cough, chest pain, breathing difficulty, pericardial, and bilateral pleural effusions secondary to mediastinal T-lymphoblastic lymphoma. Pleural fluid cytology slides and cell block sections showed numerous single dispersed neoplastic lymphoblasts with occasional giant multinucleated mesothelial cells with emperipolesis of lymphocytes. The background showed scattered and clumped apoptotic karyorrhexis debris and reactive mesothelial cells. Cell block immunohistochemistry showed CD3, CD5, CD7, CD10, CD99, and TdT positive lymphocytes, consistent with involvement by T-lymphoblastic lymphoma. The giant cells were positive for cytokeratin, calretinin and WT1 confirming their mesothelial origin. Lymphoid effusions with emperipolesis may raise a potential diagnostic pitfall because they may morphologically be confused with other inflammatory and neoplastic lesions. This cell-in-cell phenomenon can be a helpful clue in the differential diagnosis of lymphocyte-rich effusions since it has been described in association with lymphomas. It might shed some light on the lymphocyte-mesothelial interaction and the potential phagocytic antigen-presenting properties of mesothelial cells under certain circumstances.
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PMID:Emperipolesis of lymphocytes by mesothelial cells in pleural effusion involved by T-lymphoblastic lymphoma. 3262 36