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) is a disorder of unknown origin which is only infrequently associated with lymphoid neoplasms. We report the first two cases of Hodgkin's disease with simultaneous SHML-like changes in the same lymph node.
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PMID:Hodgkin's disease and sinus histiocytosis with massive lymphadenopathy-like changes. 191 95

We report a case of a 31-year-old HIV infected black female, who presented with asymptomatic generalized lymphadenopathy. Three particularly enlarged lymph nodes were biopsied (2 cervical and 1 axillary). The histological picture was consistent with a diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease. Large histiocytes, positive for a variety of macrophage markers and for the S-100 protein, were observed in the distended sinuses. A few hyperplastic follicles, such as usually seen in HIV-infection-associated lymphadenopathy, were present at the periphery of one lymph node. No infections agent besides HIV could be detected by histological or microbiological analysis or by in situ hybridization. This is the first reported case of SHML associated with HIV infection. The possible relationship between the two diseases is discussed.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease) in an HIV-positive patient. 192 67

Sinus histiocytosis with massive lymphadenopathy (SHML), introduced in 1969 by Rosai and Dorfman, is a benign and self-limited (albeit often relapsing) pseudolymphomatous entity with characteristic histopathologic features. Although the presence of massive cervical lymphadenopathy often aids in the diagnosis of SHML, the disease may present in the absence of detectable nodal involvement (Rosai-Dorfman disease). Therefore, the physician must be aware of such extranodal presentations. A recent review of the 365 cases in the SHML registry has revealed a preponderance of eyelid and/or orbital involvement; only 26 cases were found with subcutaneous or soft tissue involvement in areas other than the eyelid and/or orbit. This is a case report of one such atypical presentation of SHML without clinically apparent lymphadenopathy or eyelid or orbital involvement.
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PMID:Rosai-Dorfman disease involving the premaxilla. 151 61

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare idiopathic proliferative disorder of histiocytes with characteristic morphologic features. We describe a case of SHML occurring in a 14-yr-old black male, in whom the diagnosis was made by fine-needle aspiration of a cervical lymph node. Cytologic features included increased numbers of large histiocytes associated with a polymorphous, cellular background composed of benign lymphocytes, plasma cells, and polymorphonuclear leukocytes. Lymphophagocytosis was a prominent feature. Extranodal involvement of the larynx was documented by surgical biopsy; there was no other extranodal disease. One year after diagnosis, the patient is clinically well, with no residual lymphadenopathy.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): diagnosis by fine-needle aspiration. 195 31

A case of Rosai-Dorfman disease of the uterine cervix is described. The disease is characterized by a mixed chronic inflammatory cell infiltrate in which histiocytic cells predominate. A characteristic feature is the presence within many of the histiocytic cells of engulfed, but undamaged, lymphocytes. Rosai-Dorfman disease is of unknown etiology and pursues a benign course. The disease can occur at a wide variety of extranodal sites, but this appears to be the first reported instance of female genital tract involvement.
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PMID:Rosai-Dorfman disease of the uterine cervix. 203 69

The histiocytoses, whether reactive or neoplastic, can be related histologically and immunophenotypically to their normal counterparts within the histiocytic system. This system has two subsets: The dendritic (antigen-presenting) cells and the phagocytic histiocytic (antigen-processing) cells. Dermatopathic lymphadenitis and Langerhans cell histiocytosis (histiocytosis X) are reactive proliferative disorders of dendritic cells. Malignancies of dendritic cells exist, but they are very rare. Benign proliferations of phagocytic histiocytes include the hemophagocytic syndromes, both familial and reactive, as well as sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and histiocytic necrotizing lymphadenitis (Kikuchi's disease). Neoplasms of phagocytic histiocytes include acute monocytic leukemia and the very rare entities, malignant histiocytosis and true histiocytic lymphoma. The latter must be distinguished from sinusoidal, large cell anaplastic lymphomas.
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PMID:The histiocytoses: clinical presentation and differential diagnosis. 215 Mar 25

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a unique disease of unknown etiology with a childhood predilection. Reports have established the worldwide distribution of the disorder. Although peripheral lymphadenopathy is the most common mode of presentation, numerous studies and individual case reports have established the fact that 30-40% of affected individuals have extranodal manifestations particularly in the head and neck region where the adenopathy tends to be concentrated. As yet, the pathogenesis of SHML has not been established but speculation relates the disorder to an aberrant response to an unspecified antigen, possibly an infectious organism. Because of the clinical manifestations, the radiographic features of SHML are not pathognomonic but rather engender a differential diagnosis which includes lymphomatous, pseudolymphomatous and infectious conditions. Appropriate imaging of patients with SHML depends upon presenting symptoms and signs. Radionuclide bone scanning may be helpful in the evaluation of suspected skeletal lesions or joint symptoms. Gallium scanning is often positive in nodal disease and CT, MRI and sonography are helpful in the evaluation of extranodal sites of involvement such as the orbit, eyelid, upper aero-digestive tract and retroperitoneum.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). 220 71

Sinus histiocytosis with massive lymphadenopathy is a benign disorder often characterized by spontaneous remission. Although immunologic abnormalities are common and high Epstein Barr virus titers have been reported, to date no case has been associated with malignant lymphoma. The authors report here the first case of this association.
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PMID:Sinus histiocytosis with massive lymphadenopathy and malignant lymphoma. An unreported association. 229 67

The production of carcinoembryonic antigen (CEA) by human breast cancer tissue has been studied in relation to the prognosis of 60 patients with breast cancer who were classified in Clinical Stage of UICC I, II and III, and who were treated by radical mastectomy. Tissue CEA was studied in primary tumors using an immunoperoxidase (PAP) method. The obtained results were as followings: 1) Patients who had CEA-negative tumors had significantly higher 10-year survival rates. 2) The positive staining rate of CEA rises as Clinical Stage and metastatic status of lymph nodes advanced. 3) There were significant correlations between staining rate of CEA and classification of histological type and differentiation. 4) There were significant correlations between staining rate of CEA and lymphoid infiltration around main tumor and Sinus Histiocytosis in regional lymph nodes. 5) These results suggest that immunohistological assessment of CEA in breast cancer tissue may provide more precise prognostic information.
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PMID:[A clinicopathological study on carcinoembryonic antigen in breast cancer]. 237 Apr 34

We report the histological findings seen in the lymph nodes draining the sites of large joint prostheses. Two patients underwent multiple prosthetic joint replacements. In one patient, the regional lymph nodes were enlarged during the revision of a total hip prosthesis, and a representative lymph node was resected. The other patient had undergone a pelvic lymph node dissection as part of a staging procedure for prostatic carcinoma. By light microscopy, the lymph nodes from both patients showed markedly dilated nodal sinuses filled with macrophages containing abundant eosinophilic, PAS-positive, granular material. Polarization microscopy revealed needle-like particles within the cytoplasm of the macrophages. We believe that the histological appearance of the lymph nodes represents a florid foreign body reaction to fragments of polyester or polyethylene derived from the articulating surfaces of the joint prostheses and transported to the regional lymph nodes via the lymphatic circulation. Sinus histiocytosis seen in the lymph nodes draining the sites of joint prostheses may resemble, and must be distinguished from, other conditions invoking a sinus pattern of lymphadenopathy, as well from benign and malignant diseases that involve the lymph nodes in a pattern mimicking sinus histiocytosis.
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PMID:Changes seen in lymph nodes draining the sites of large joint prostheses. 259 17


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