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)

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a benign, self-limited pseudolymphomatous entity with characteristic histologic features. The disease may be atypically present in the absence of detectable nodal involvement. This is a case of nasal septum involvement of Rosai-Dorfman disease without clinically apparent lymphadenopathy.
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PMID:Rosai-Dorfman disease involving the nasal septum. 855 83

Rosai-Dorfman syndrome of Sinus Histiocytosis with Massive Lymphadenopathy is a benign disorder of unknown etiology which is characterized by proliferation of phagocytic-like cells that infiltrate tissues. We present here a case of a 17 years old boy with the enlargement of the cervical lymph nodes who developed this disease. The diagnosis was established by the histopathological examination. There was no evidence of recurrence during the 18 months of follow up.
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PMID:[Rosai-Dorfman syndrome in a 17 year old boy as a diagnostic problem of lymphadenopathy]. 857 45

Sinus histiocytosis with massive lymphadenopathy is an entity that exceptionally appears as a purely cutaneous disease. We described 2 cases of this form with spontaneous involution. Histology showed a polymorphic infiltrate in the dermis and hypodermis composed of large histiocytes with lymphophagocytosis, neutrophils and plasma cells. Immunohistochemical stains demonstrated the positivity of histiocytes for protein S-100 and MAC 387.
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PMID:Purely cutaneous Rosai-Dorfman disease. 858 84

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare entity characterized by the proliferation of cells belonging to the macrophage-histiocyte family, but whose exact origin is unknown. A case of SHML occurring in a 62-yr-old female is described in which the diagnosis is initially suggested by fine-needle aspiration (FNA) of a cervical node. The smears show a background of lymphocytes and plasma cells and large histiocytes with well-preserved lymphocytes in their cytoplasm (emperipolesis or lymphophagocytosis). Both nodal and extranodal (nasal mass) involvement are confirmed by surgical biopsy. The immunohistochemistry suggests that SHML cells are functionally activated macrophages. Eight months after diagnosis, the patient is clinically well, with partial improvement of lymphadenopathy.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): diagnosis with fine-needle aspiration in a case with nodal and nasal involvement. 909 55

Sinus histiocytosis with massive lymphadenopathy is an uncommon disease affecting individuals of either sex and of all ages and ethnic backgrounds. We present a case of sinus histiocytosis confined to the skin in a 65-year-old woman whose diagnosis was coincident with herpes simplex virus infection and polybacterial urinary infection, both asymptomatic. The polymerase chain reaction for herpes simplex virus and human herpesvirus-6 from involved skin was negative. We review previously published cases of sinus histiocytosis limited to the skin.
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PMID:Sinus histiocytosis confined to the skin. Case report and review of the literature. 860 Aug 4

Rosai-Dorfman disease is a rare, idiopathic, benign histiocytic proliferation usually seen in younger patients. Massive lymphadenopathy most commonly involves the cervical lymph nodes, with a predominant infiltration of sinusoidal histiocytes. Nearly half of the patients will have extranodal involvement, 75% occurring in sites in the head and neck. Three cases of extranodal Rosai-Dorfman disease of the head and neck involving the nose, paranasal sinuses, and parotid gland are presented. The clinical presentation, histologic characteristics, radiographic findings, and treatment of the disease are discussed. Because of the scarcity of cases, the clinical and histopathologic features of this disease may be overlooked. Familiarity with its relatively frequent clinical manifestations in the head and neck, as well as with the diagnostic histopathology, should preclude confusion with other disease entities.
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PMID:Extranodal Rosai-Dorfman disease of the head and neck. 861 84

A case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman syndrome) in a 42-year-old man is described. The disease involved the respiratory tract and skeletal apparatus, led to considerable general impairment and was unusually responsive to glucocorticoids. Molecular and immunophenotype analysis seem to confirm the reactive nature of a disorder that shows profound T-cell immunodeficiency.
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PMID:Rosai-Dorfman syndrome with extranodal localizations and response to glucocorticoids: a case report. 864 49

Langerhans' cell histiocytosis (LCH) is a clonal proliferation of Langerhans cells (LC) showing histologically an abundant reactive infiltrate composed of macrophages and lymphocytes, as well as eosinophilic and neutrophilic granulocytes. Rosai-Dorfman disease (RDD) shows a sinusoidal accumulation of large histiocytic cells with an immunophenotype similar to LC of LCH. The histological picture of LCH is reminiscent of an inflammatory disorder and LC may produce cytokines and are influenced by these soluble factors. This study set out to establish the monokine expression pattern in LCH in comparison with those of RDD; dermatopathic lymphadenopathy, which also shows a proliferation of S100-positive dendritic cells; and LC in normal skin specimens. Isotopic in situ hybridization was used for the detection of transcripts of tumour necrosis factor-alpha (TNF-alpha), interleukin-6 (IL-6), and IL-1 beta, in some cases combined with immunohistology for the S100 protein or CD68. In all 11 tissue samples from eight patients, LC of LCH expressed TNF-alpha; in two cases IL-1 beta transcripts were additionally noted in some LC, whereas IL-6 was found in reactive cells. Large histiocytic cells of RDD expressed all three monokines, whereas minimal or no expression of these cytokines could be detected in interdigitating reticulum cells in dermatopathic lymphadenopathy. In two out of five normal skin samples, only TNF-alpha specific signals were observed in LC. These data suggest that histologically different lesions of the histiocytic/dendritic cell system display distinct cytokine profiles. The expression of monokines, which have been demonstrated to influence various functions of epidermal LC, may play a role in the pathogenesis of LCH. Systemic symptoms in RDD may be related to enhanced production of monokines in these lesions.
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PMID:Monokine expression in Langerhans' cell histiocytosis and sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) 912 Jul 35

We report a 3-year-old girl presenting with bilateral cervical lymph node enlargement persisting for > 3 months. Leukocytosis, elevated erythrocyte sedimentation rate, a marked hypergammaglobulinemia, and a moderate hepatosplenomegaly were also found. The diagnosis of sinushistiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, was established histologically by the demonstration of characteristic sinushistiocytosis with lymphocytophagocytosis. Treatment was started with high dose steroids, and a decline of lymph node size and a normalization of laboratory parameters occurred. However, when steroids were tapered, lymph node size rapidly reincreased. Chemotherapeutic treatment was started using etoposide, which was completely ineffective. Therefore, treatment was changed to a combinatory low dose methotrexate therapy and 6-mercaptopurine for 4 months. Whereas a prompt and complete remission was reached, single 6-mercaptopurine therapy was maintained and treatment has been discontinued after a total of 2 years. The child has remained healthy for 7 years. This case would recommend the use of methotrexate and 6-mercaptopurine for treatment of complicated SHML.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): response to methotrexate and mercaptopurine. 869 98

Cutaneous lesions of Rosai-Dorfman disease (RDD) are usually associated with nodal or other extranodal localization. We describe a female patient with RDD clinically limited to the skin. The patient presented with asymptomatic red-brown papules and nodules on the legs, arms, back, and nose. Histologically, the lesions consisted of a proliferation of large histiocytes occasionally showing emperipolesis. Histiocytes were also observed within dilated lymphatic vessels. Immunohistochemical study showed that histiocytes expressed S-100 protein and both macrophage and monocyte markers. All lesions resolved completely with Roentgen therapy. No recurrence has been observed over a 3-year follow-up period.
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PMID:Purely cutaneous Rosai--Dorfman disease. 873 85


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