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) 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.
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PMID:Sinus histiocytosis with massive lymphadenopathy and epidural involvement. 732 32

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) is a rare, but well-documented entity. We report a male patient who presented with progressive paraparesis, with thoracolumbar extradural lesion (from D11 to L2 level) on magnetic resonance imaging (MRI). He underwent D12-L2 laminectomy followed by total removal of extradural spinal space-occupying lesion (SOL). Histopathological diagnosis of the lesion was RDD. Four weeks after surgery, he was treated with external beam radiotherapy, total dose: 50.4GY in 28 fractions. On three-month follow-up, he did not have any neurological deficits. There was no evidence of other extranodal or lymph node involvement. This case has been reported on account of rare presentation of this disease as spinal extradural lesion. Pertinent literature has been reviewed.
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PMID:Extranodal Rosai-Dorfman Disease presenting as spinal extradural lesion: a case report with a review of the literature. 2336 Dec 92

A 19-year-old woman presented with a history of severe lower backache and asymmetric proximal lower limb weakness during the past 3 months. In addition, she also suffered from lower motor neuron-type bladder and bowel symptoms. On examination, paraparesis was noted. Further, sensory examination suggested patchy asymmetric sensory loss in both lower limbs with saddle anaesthesia and areflexia. A clinical diagnosis of Conus-Cauda syndrome was made and contrast-enhanced MRI of the lumbar and sacral spine was done, which confirmed the presence of a mass lesion within the spinal canal involving the cauda equina extending up to the sacral level. She underwent partial resection of the lesion following which the neurological deficits and lower backache resolved. Histopathological evaluation and immunohistochemical analyses uncovered Rosai-Dorfman disease. There was no evidence of disease elsewhere in the body. Since the patient improved significantly following surgery and exhibited no further neurological worsening, she remains under close follow-up.
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PMID:A quintessential syndrome with a rare marvelling aetiology: Rosai-Dorfman disease presenting as Conus-Cauda syndrome. 2945 79

Rosai-Dorfman disease (RDD) predominantly affects cervical lymph nodes and presents with fever and pancytopenia. Central nervous system involvement though uncommon is often reported. Hydrocephalus and paraparesis as a consequence of RDD is an extremely rare entity. We present a 58-year-old male, diagnosed and treated for RDD with cervical lymphadenopathy, who now presented with spastic paraparesis and on evaluation was found to have communicating hydrocephalus that resolved after ventriculoperitoneal shunt surgery.
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PMID:Spastic Quadriparesis and Communicating Hydrocephalus as Late Sequel of Rosai-Dorfman Disease: A Case Report and Review of Literature. 2968 50