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)

A 45-year-old woman had pyrexia, headaches, collapse and hyponatraemia. Intracerebral abscess, bacterial meningitis and subarachnoid haemorrhage were excluded. She was given intravenous antibiotics and gradually recovered. One month later she was readmitted with diplopia, headache and vomiting. Serum sodium was low (107 mmol/l) and a diagnosis of inappropriate ADH secretion was made. MRI scan showed a suprasellar tumour arising from the posterior pituitary gland. A skin rash gradually faded. Serum cortisol, prolactin, gonadotrophins and thyroid hormone levels were low. A pituitary tumour was removed trans-sphenoidally, she had external pituitary radiotherapy, and replacement hydrocortisone and thyroxine. She was well for 12 months when she developed progressive weakness and numbness of both legs. Examination suggested spinal cord compression at the level of T2 where MRI scanning showed an intradural enhancing mass. This spinal tumour was removed and her neurological symptoms disappeared. Nine months after this she developed facial pain and nasal obstruction. CT scan showed tumour growth into the sphenoid sinus and nasal cavities. A right Cauldwell-Luc operation was done and residual tumour in the nasal passages was treated by fractionated external radiotherapy and Prednisolone. Histological examination of the specimens from pituitary, spinal mass, and nasal sinuses showed Rosai-Dorfman disease, a rare entity characterized by histiocytic proliferation, emperipolesis (lymphophagocytosis) and lymphadenopathy. Aged 48 she developed cranial diabetes insipidus. Although Rosai-Dorfman syndrome is rare, it is being reported with increasing frequency, and should be borne in mind as a possible cause of a pituitary tumour.
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PMID:Rosai-Dorfman disease presenting as a pituitary tumour. 1034 67

General anesthesia in patients with mediastinal masses can lead to life-threatening cardiorespiratory complications. We report the cases of 2 pediatric patients with mediastinal masses who developed serious complications during general anesthesia. The first was a 13-year-old boy with a suspected diagnosis of high-grade T-cell non-Hodgkin lymphoma, lymph node disease, and an anterior mediastinal mass. He developed negative pressure pulmonary edema secondary to severe upper airway obstruction in spontaneous ventilation. The second was a 14-year-old boy with Rosai-Dorfman disease and paratracheal lymph node involvement. He developed severe airway obstruction in the early postoperative period. The anesthetic difficulties that arise in these cases include acute airway occlusion, superior vena cava syndrome, pulmonary artery or cardiac compression, acute pulmonary edema, and cardiopulmonary collapse. The technique of choice is induction with inhaled anesthetics and maintenance of spontaneous ventilation. Neuromuscular relaxants are avoided.
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PMID:[Perioperative respiratory complications in pediatric patients with mediastinal masses: a report of 2 cases]. 1730 81

Sinus histiocytosis with massive lymphadenopathy known as Rosai-Dorfman disease is characterized by painless bilateral cervical lymphadenopathy. Extranodal manifestations are uncommon and spinal involvement is rare. A 15-year-old man presented with intermittent midthoracic back pain only. He had no specific findings on neurologic examinations, hematologic and biochemical laboratory tests. Radiological examination of thoracic spine revealed collapse of T6 vertebrae with thoracic kyphosis and osteolytic lesion of T12 vertebra body. He underwent a removal of bone tumor, anterior reconstruction with mesh and pedicle screw fixation via posterior approach for pathologic confirmation and stabilization. Histopathologic study of the lesion revealed focal infiltration of large histiocytes showing emperipolesis. Immunochemistry stain of histiocytes was positive for CD68 and S-100 but negative for CD1a. This report presents a rare case and literature review of extranodal Rosai-dorfman disease in thoracic spine.
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PMID:Rosai-dorfman disease in thoracic spine: a rare case of compression fracture. 2534 69

Rosai-Dorfman disease is a rare benign histiocytic disorder that arises predominantly in lymph nodes with fever and malaise, but can affect various organs, with or without lymphadenopathy. Solitary extranodal skeletal lesions are extremely rare. Herein, we describe a case of isolated disease of thoracic spine, with vertebral body fracture. A 14-year-old girl presented with cervical back pain for about one month as the only symptom. There was no lymphadenopathy. Results of laboratory tests were within reference ranges. The radiological examinations revealed collapse, osteolytic lesion and compressive fracture, which reduced vertical diameter of C4 vertebral body. The patient made a complete recovery after a limited C4 laminectomy, spondylodesis C3/C5 vertebra and biopsy of the lesion. Histologically, nonspecific cellular milieu and atypical histiocytic proliferation with emperipolesis, with CD68 and S100 positivity, confirmed a diagnosis of Rosai-Dorfman disease. Isolated extranodal skeletal Rosai-Dorfman disease should be considered among the differential diagnoses of similar changes, such as Langerhans cell histiocytosis, Erdheim-Cheister disease, eosinophilic granuloma, osteomyelitis, metastasis and lymphoma.
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PMID:Rosai-Dorfman disease of vertebra: Case report and literature review. 2862 Nov 4

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare and benign disease that usually presents as massive and painless cervical lymphadenopathy. We are reporting this rare disease with systemic manifestations and causing internal jugular vein thrombosis and middle lobe collapse-consolidation.
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PMID:Rosai-Dorfman disease presenting as internal jugular vein thrombosis and middle lobe collapse-consolidation. 3288 5