Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
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) was recognized as a new clinical-pathological entity in 1969. Up to the present 134 cases have been described. The disease is characterized by prominent cervical lymph node enlargment. Microscopic features include marked dilatation of sinuses with intrasinusal histiocytes and lymphophagocytosis. About 70% of the patients reported were affected during the first 2 decades of life. The disease is held to be benign on account of spontaneous resolution in some patients. A follow-up survey of 72 patients showed disappearance of the symptoms 10 years after the original diagnosis in 24 patients. In 42 patients the disease still persisted 6 months to 21 years later. Six patients died, but only one of them as a result of the disease. Extranodal involvement was seen in the orbit, eyelid, respiratory tract, skin, bone, salivary glands, and testis. In two cases, one of which will be reported here, paraparesis resulted from infiltration of the epidural space. Treatment with prednisolone was tried in some cases with excellent results. In our case treatment with prednisolone and vinblastine resulted in the disappearance of the neurological symptoms.
 ...
PMID:Sinus histiocytosis with massive lymphadenopathy and epidural involvement. 732 32

A re-examination was made of bioptic investigations of lymph nodes in a group of patients with spinocellular carcinomas of the oral cavity. With regard to the patients' survival, lymphocytic predominance and sinus histiocytosis appear to be changes with the most favourable prognosis. The predominance of embryonic centres of follicles cannot, in the author's group, be interpreted as signs of better prognosis. Sinus histiocytosis was never found in nodes with metastases.
 ...
PMID:[Regional lymph nodes in carcinoma of the oral cavity]. 743 41

Selected aspects of the histopathology of Langerhans cell histiocytosis representing diagnostic difficulty and/or controversy are presented with emphasis on the composition of pathological lesions. Lesional cell phenotypes and the factors influencing variations are noted. Features of several skin-based histiocytic disorders, dermatopathic lymphadenopathy and Rosai-Dorfman disease are compared. Associations between Langerhans cell histiocytosis and juvenile xanthogranuloma and malignant disorders are considered. Observations of potential significance in the eventual elucidation of the pathogenesis of these enigmatic diseases are presented.
 ...
PMID:The histopathology of Langerhans cell histiocytosis. 752 Dec

Sinus histiocytosis with massive lymphadenopathy (SHML) is a condition of unknown aetiology characterized by proliferation of histiocytes and lymphocytes, with the former phagocytosing the latter producing a typical histological appearance. Although first described in lymph nodes, cases have been reported in many extranodal sites, including the central nervous system. We report a case of SHML involving the sella turcica producing diabetes inspidus and pituitary dysfunction.
 ...
PMID:Sinus histiocytosis with massive lymphadenopathy localized to the sella. 757 85

Rosai-Dorfman disease is a rare idiopathic histioproliferative disease affecting the lymph nodes. Extranodal involvement has also been recognized, but central nervous system manifestations are extremely rare. Only 12 patients with intracranial involvement have been reported previously, and they all presented with clinical and radiological findings suggestive of meningioma. We report multiple meningeal nodules in a patient presenting with seizures whose pathological findings at surgery confirmed the diagnosis of Rosai-Dorfman disease. A review of all previously reported intracranial lesions is presented.
 ...
PMID:Rosai-Dorfman disease mimicking multiple meningioma: case report. 764 1

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.
 ...
PMID:Langerhans' cell histiocytosis (histiocytosis X): immunophenotype and growth fraction. 769 Jul 35

NBXFO hybridoma cells produced macrophage colony-stimulating factor (M-CSF), which stimulated the growth of murine bone marrow-derived macrophages with potent suppressor activity. These macrophages suppressed lymphocyte responses to mitogens and antigens in a dose-dependent manner. Using a transwell chamber, we demonstrated that macrophages needed physical contact with the lymphocytes to suppress lymphocyte proliferation on day 1 in the concanavalin A mitogen reaction. In addition, no soluble suppressor factor was detected at that time. The number of lymphocytes disappeared with time when they were cocultured with the macrophages. Electron microscopy revealed that the macrophage phagocytosized the lymphocytes after 7 1/2 h. Dextran sulfate, heparan, and fucoidan prevented the macrophages from suppressing the lymphocytes. This phenomenon resembles the human disease sinus histiocytosis, also called Rosai-Dorfman disease, in which macrophages (histiocytes) phagocytosize autologous lymphocytes; occasionally, this disease is associated with immunological abnormalities. Thus we believed that macrophage-activating cytokines, such as M-CSF, may stimulate macrophages to phagocytose lymphocytes in vivo.
 ...
PMID:Macrophage colony stimulatory factor-activated bone marrow macrophages suppress lymphocytic responses through phagocytosis: a tentative in vitro model of Rosai-Dorfman disease. 779 Jul 77

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a non-hereditary histiocytic proliferative disorder affecting young people, with extranodal manifestations in 28-43% of cases. Eye involvement is infrequent. Lymphoproliferation in the soft tissues of the orbit and in the lids has been reported in 12% of cases but intraocular involvement is rare. We describe the case of a 12-year-old boy affected by Rosai-Dorfman disease with bilateral relapsing uveitis and papilledema that appeared four years before the onset of lymphadenopathy.
 ...
PMID:Relapsing bilateral uveitis and papilledema in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). 779 3

Cutaneous Rosai-Dorfman disease in a male patient presented as a clinically malignant left breast mass. The intra-operative frozen section indicated a benign inflammatory lesion and a simple excision was performed. The disease was apparently limited to the skin and subcutis of the left breast with no lymph nodes or other organs involvement. While the lesion demonstrated the characteristic morphology of Rosai-Dorfman disease, the diagnosis of granulomatous mastitis, epithelioid sarcoma and other cutaneous xanthohistiocytic proliferations were considered. In the absence of lymphadenopathy, a high index of diagnostic awareness is required to recognize the uncommon, solitary cutaneous Rosai-Dorfman disease.
 ...
PMID:Cutaneous Rosai-Dorfman disease presenting as a suspicious breast mass. 779 96

Retrospective analysis of childhood histiocytoses treated at the Department of Pediatrics, Ramathibodi Hospital from May 1970 till June 30, 1992 to verify the prevalence according to the recent classification, course and prognosis was conducted. Among 120 cases, 54 were Class I or Langerhan Cell Histiocytosis (LCH, previously called Histiocytosis-X). Nineteen cases were Class II: Infection-Associated Hemophagocytic Syndrome (IAHS) and Sinus Histiocytosis. Forty-seven cases were class III which included acute monocytic leukemia, juvenile CML; malignant histiocytosis, HMR, and histiocytic lymphoma. Excellent prognosis (cure) was seen in all cases of Hand-Schuller Christian Disease, eosinophilic granuloma, sinus histiocytosis with massive lymphadenopathy and many cases of Class II (except IAHS). The worse prognosis (100% mortality rate) was seen in HMR and juvenile CML. The intermediate prognosis (50%, 54%, 58.8% and 66.7% mortality rate) was seen in MH, HL, LSD and IAHS respectively. To differentiate between IAHS and MH/HMR, the prominent bone marrow findings in 12 cases of IAHS revealed that every case showed prominent hemophagocytosis by the promono-histiocytes/histiocytes, the maximal total erythroblasts (TE) were only 7.5 per cent except for 3 cases; in which one case with agranulocytosis from co-trimoxazole had 84 per cent erythroid cells, one case with prior co-trimoxazole treatment had 37 per cent TE, the last one had 40 per cent TE with massive GI bleeding while in the recovery stage of DHF. All 5 cases of HMR had prominent hemophagocytosis and increased TE (> 22%).(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Histiocytoses in children: analysis of 120 cases and the bone marrow findings in infection-induced hemophagocytic syndrome vs malignant histiocytosis. 782 9


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>