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)

We report on a patient who, at 31 years of age, was found to suffer from sinus histiocytosis with massive lymphadenopathy (SHML; Rosai-Dorfman disease) with nodal and extranodal involvement as described previously. Five years later the patient presented with nephrotic syndrome caused by a generalized AA amyloidosis, and he subsequently died from pulmonary thromboembolism owing to renal vein thrombosis. Retrospective analysis of serum levels of C-reactive protein (CRP) showed that during the last 3 years before his death, he had a persistently elevated CRP level ranging from 73 to 161 mg/L, despite antiinflammatory treatment with prednisolone, methotrexate, or 6-mercaptopurine. These figures indicate that the patient was probably suffering from a permanent acute phase response which, in the absence of any other evidence of a chronic inflammatory disease which commonly causes AA amyloidosis, was most likely owing to SHML.
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PMID:Rosai-Dorfman disease and generalized AA amyloidosis: a case report. 1083 4

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare but distinct clinicopathologic entity characterised histologically by a benign s histiocytic proliferation. Isolated involvement of extranodal sites without concomitant nodal disease is rare. We describe the pathological features of 2 cases of Rosai-Dorfman disease that were clinically confined to the skin. In both male adult Chinese patients, proliferation of histiocytes was accompanied by S-100 protein expression demonstrated immunohistochemically within the histiocytes. The pathology of Rosai-Dorfman disease and its microscopic differential diagnoses are discussed.
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PMID:Cutaneous Rosai-Dorfman disease--a pathologic review of 2 cases. 1106 96

Sinus histiocytosis with massive lymphadenitis or Rosai-Dorfman disease (RDD) is a rare, benign, proliferative histiocytic disease of unknown origin. It predominately affects the lymph nodes The skin is the extranodal organ most frequently involved, although a few patients present with only lesions of the skin and soft tissues, without involvement of the lymph nodes (purely cutaneous RDD). Ocular involvement is rare, and generally localized to the eyelid. We report an unusual case of cutaneous sinus histiocytosis without nodal involvement and with bilateral uveitis in a girl. As far as we know, this is the first case reported in which cutaneous and ocular RDD occurred without nodal involvement.
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PMID:Cutaneous sinus histiocytosis and chronic uveitis. 1108 66

Sinus histocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Disease (RDD), is an idiopathic histiocytic proliferation affecting lymph nodes. Although extranodal involvement has been reported in diverse sites, central nervous system (CNS) manifestation, particularly in the absence of nodal disease is uncommon. We report 11 cases of RDD primary to the CNS without evidence of other sites of involvement. The cases included 7 males and 4 females ranging in age from 22 to 63 years (mean: 41 y). The patients presented with headaches, seizures, numbness, or paraplegia. Eight cases involved the cranial cavity and three cases, the spinal canal. Lesions were most often extra-axial and dura based. Only one presented in the CNS parenchyma. Histologically, the lesions consisted of variable numbers of pale-staining histocytes with emperipolesis often overshadowed by extensive lymphoplasmacytic infiltrates and fibrosis in the background. Special stains for organisms were negative. By immunohistochemical analysis, the characteristic histiocytes were positive for S100 protein and CD68 and negative for CD1a. Treatment consisted of surgical biopsy or excision. Follow-up, available for 10 cases with intervals ranging from 5 days to 42 months (mean: 15 mo), disclosed one patient dying of operative complications 5 days after biopsy and nine patients with no evidence of disease progression RDD should be considered in the differential diagnosis of inflammatory lesions of the CNS. Our study suggests that this entity may have been misdiagnosed in the past as plasma cell granuloma or inflammatory pseudotumor.
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PMID:Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. 1126 22

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy), is a rare benign histiocytic proliferative disorder. Over 650 cases have been reported since 1969. To the best of our knowledge, there have been only 31 cases with central nervous system involvement reported in the literature. Intracranial disease usually presents clinically and radiologically as a "meningioma". It can be misdiagnosed as a nonspecific inflammatory process because of the atypical histologic features of Rosai-Dorfman disease occurring in a non-nodal location. Familiarity with such atypical histologic features and appropriate use of immunohistochemical stains is required for a definitive diagnosis of central nervous system Rosai-Dorfman disease. We report such an intracranial lesion with other extranodal sites of involvement with a 5-year follow up and a review of previously reported cases. Ann Diagn Pathol 5:96-102, 2001.
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PMID:A report of intracranial Rosai-Dorfman disease with literature review. 1129 95

We report a patient with sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) who presented with widespread nodal and extranodal involvement affecting the skin, orbits and nasal sinuses, complicated by the development of autoimmune haemolytic anaemia. The aetiology and pathogenesis of this multisystem disorder are unknown but are thought to represent a reactive histiocytic process to an infective agent rather than a neoplastic or other primary condition. Prognosis is generally good but clinical or laboratory evidence of immune dysfunction tends to predict a poorer outcome. We describe the clinical course of the patient and review the literature on this disease.
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PMID:Rosai-Dorfman disease complicated by autoimmune haemolytic anaemia: case report and review of a multisystem disease with cutaneous infiltrates. 1153 2

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is an unusual clinical entity. It classically presents with massive cervical lymphadenopathy, but atypical presentations have been described in the absence of nodal involvement, making diagnosis difficult. We report a case that presented with isolated involvement of the maxillary alveolus and discuss the diagnostic dilemmas and approaches to management in patients with atypical clinical features.
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PMID:Intraoral presentation of Rosai-Dorfman disease: a case report and review of the literature. 1214 77

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a rare but distinctive entity. It was initially described as a nodal-based disease but later found to affect a variety of extranodal sites. The diagnosis of Rosai-Dorfman disease is based on the unique histologic changes that are characterized by proliferation of S-100 protein-positive histiocytes, the presence of emperipolesis, and a mixture of prominent lymphoplasmacytic infiltrates. The etiology of Rosai-Dorfman disease is unknown. It usually runs a benign clinic course and may resolve spontaneously, but cases running protracted courses have also been reported. We report an unusual case of recurrent extranodal Rosai-Dorfman disease in nasal and paranasal cavities with intracranial involvement surviving for more than 9 years.
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PMID:Nasal Rosai-Dorfman disease with intracranial involvement: a case report. 1276 7

A 56-year-old woman with vertex headaches, new-onset seizure disorder, nasal obstruction, and hyposmia was found to have a dural-based intracranial mass as well as bilateral masses arising from the inferior turbinates and extending into the nasopharynx. The patient underwent endoscopic resection of the involved portions of the inferior turbinates, including the sizable nasopharyngeal component. Craniotomy was performed at a later date. Both pathologic specimens were consistent with extranodal Rosai-Dorfman disease. The patient showed resolution of all symptoms after surgery. Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is a rare entity usually presenting as cervical nodal disease. When extranodal disease of the head and neck is present, prompt recognition and conservative surgical management is the treatment of choice for this benign pseudolymphomatous entity.
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PMID:Rosai Dorfman disease presenting as synchronous nasal and intracranial masses. 1460 79

Rhinoscleroma is an uncommon chronic, destructive infection of the respiratory mucosa caused by Klebsiella rhinoscleromatis. This coccobacillus can be found in the typical histiocytes, the Mikulicz cells. Extranasal and nodal involvement in this disease is rare, but documented. Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is also a rare, non-hereditary disorder. Bilateral cervical lymphadenopathy with emperipolesis, as the main histological characteristic, is the most common presentation. It can also occur extranodally. We report a case of rhinoscleroma occurring in a 62-year-old woman since 1984, who developed parotid gland and lymph node involvement. The changes in the nasal mucosa and the parotid gland showed chronic inflammation with Mikulicz cells. In the lymph nodes, features characteristic of Rosai-Dorfman disease were seen. Taking into consideration the literature dealing with both of these diseases, we discuss that Rosai-Dorfman disease could be a special type of lymph node reaction and is not necessarily an entity of its own. Therefore, it should be known as Rosai-Dorfman lymph node reaction. Furthermore, there seems to be an interconnection between Rosai-Dorfman disease and rhinoscleroma.
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PMID:Rhinoscleroma associated with Rosai-Dorfman reaction of regional lymph nodes. 1472 Jan 40


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