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 (sinus histiocytosis with massive lymphadenopathy) is a rare disease characterised by benign proliferation of histiocytes, with painless lymph node enlargement and frequent extranodal involvement. Orbital involvement occurs in 11% of cases and onset of the disease is typically in the first two decades of life. A benign course is usual, but in some cases blindness or fatality may result. We present a case with late onset at age 78 years and lacrimal gland involvement, and review the literature on this disease.
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PMID:A case of Rosai-Dorfman disease involving the lacrimal gland in an elderly patient. 1202 74

We report a young adult man who presented with chronic raised intracranial tension features and unusually progressive bilateral visual and hearing impairment of 18 months duration. MR imaging showed extensive dural involvement and contiguous orbital and spinal disease. Cerebrospinal fluid demonstrated persistent high lymphocytic pleocytosis. Dural biopsy obtained from posterior cervical approach with C1 arch excision and meningeal biopsy revealed features of classical of Rosai-Dorfman disease. Histiocytes were strongly positive for CD-68 and S-100 proteins. The illness relentlessly progressed with patient developing total deafness and near total blindness at last follow-up.
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PMID:Rosai Dorfman disease: case with extensive dural involvement and cerebrospinal fluid pleocytosis. 2202 38

Background. Rosai-Dorfman disease (RDD) is a benign histiocytic proliferative disorder of unknown etiology. This rare condition commonly causes massive cervical lymphadenopathy. Intracranial RDD without any nodal involvement is extremely rare. Case Report. A young Bangladeshi male complained of bilateral complete blindness with left sided deafness for about three years. There was no lymphadenopathy. MRI and CT scan of brain suggested an inflammatory/neoplastic (?meningioma) lesion located at left parasellar region which extended frontally to encircle both optic nerves and also to left prepontine area. Histopathologically the lesion was diagnosed as RDD. The patient was treated with steroid and significant clinical improvement observed. Conclusion. The prognosis of intracranial RDD is not poor. It can be treated with surgery with or without corticosteroids, chemotherapy, and so forth. But as the condition is extremely rare and often misdiagnosed, the clinician, radiologist, and histopathologist should have a suspicion in their mind about the possibility of RDD.
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PMID:Isolated Intracranial Rosai-Dorfman Disease. 2694 55

Rosai-Dorfman Disease also called as Sinus Histiocytosis with Massive Lymphadenopathy is a rare, benign, idiopathic histiocytic proliferative disorder that occurs predominantly in children and young adults. Orbital involvement can occur in 11% of cases. Isolated lacrimal gland involvement without any local or systemic recurrence is very rare. To the best of our knowledge, only seven cases have been reported in the literature till date. Histopathological and immunohistochemical confirmation is essential in establishing the diagnosis. A benign course is usual, but in some cases, blindness or even fatality may result. We report a case of 30-year-old female with isolated lacrimal gland involvement and 19 months' follow-up.
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PMID:Rosai-Dorfman disease with isolated lacrimal gland enlargement. 3050 30