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)

Extranodal lesions may be the sole manifestation of Rosai-Dorfman disease (RDD). Although the head and neck region is one of the most common extranodal sites, laryngeal involvement is very rare. A 44-year-old woman presented with a complaint of progressive dyspnea. She had a three-year history of treatment for asthma and a history of operation for a nasal mass that afflicted her for 15 years and was diagnosed as rhinoscleroma. On physical examination, she had three subcutaneous lesions, in the left lower eyelid, right epicanthal area, and left forearm, respectively. No lymphadenopathy was present. Laryngoscopic examination revealed three solid, polypoid masses in the subglottic region, 1 cm in diameter. With a two-staged operation, the laryngeal masses were excised totally together with the subcutaneous lesions. Histological examination of all the specimens showed proliferation of histiocytes. Immunohistochemical staining revealed typical S-100 protein-positive histiocytes and emperipolesis. Both laryngeal and subcutaneous lesions were diagnosed as RDD. A re-evaluation of sections from the previous operation specimen of the nasal mass showed the same morphological features. The patient was healthy without recurrence, nine months following surgery.
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PMID:Rosai-Dorfman disease presenting as laryngeal masses. 1862 47

The present report concerns an unusual case of extranodal Rosai-Dorfman disease involving the epicardium, mesenterium, pleural cavity and lung. All involved lesions showed characteristics of S100-positive histiocytes exhibiting emperipolesis. The patient was a 51-year-old woman with a 2-year evolution of chest distress, dyspnoea and oedema. Pathological examination indicated that heart failure, which resulted from constrictive pericarditis, led to the fatal outcome of this case. Interference from severe hydropericardium prevented timely diagnosis and appropriate treatment. Therefore it is recommend that, despite being a rare condition, pericardium-involved Rosai-Dorfman disease should be taken into consideration in differential diagnoses, especially in cases of severe hydropericardium.
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PMID:Hydropericardium prevented diagnosis of constrictive pericarditis: an unusual case of Rosai-Dorfman disease. 2185 4

Rosai-Dorfman disease, or sinus histiocytosis, first described in 1969, is a benign systemic disorder of histiocytes of unknown etiology characterized by massive lymphadenopathy. It predominantly involves the head and neck region, and is often associated with extranodal involvement. Patient presented to pulmonology with history of sudden onset dyspnea. Clinically and radiologically, the lungs were normal. D-dimer test was noncontributory. As the patient had a family history of lymphedema, lymphoscintigraphy was sought. We report the detection of an unsuspected case of Rosai-Dorfman disease by lower limb lymphoscintigraphy.
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PMID:Unsuspected chylous ascites in an asymptomatic case of Rosai Dorfman disease. 2356 82

This study was performed to investigate the clinical manifestations, treatment methods, and prognosis of Rosai-Dorfman disease (RDD) with laryngeal involvement. Five clinical cases of RDD with laryngeal involvement diagnosed between 1986 and 2015 were retrospectively analyzed. The laryngeal lesions of these 5 patients mostly involved the glottis and subglottis, with the main symptoms being a hoarse voice and airway obstruction. In addition, the patients mostly exhibited a unilateral or asymmetric onset that was manifested by a laryngeal submucosal nodular mass. The patients were subjected to a regimen of hormone treatment combined with surgical resection. The median follow-up duration was 101 months (8-384 months). One case was lost, and the remaining 4 subjects are alive with disease. The follow-up examinations revealed that 4 subjects had stable laryngeal conditions, whereas one showed minor progression. RDD with laryngeal involvement is clinically rare and differs considerably from classical RDD in age of onset, gender composition, and extranodal involvement. The regimen of hormone treatment combined with surgical resection can stabilize the patient's general condition and laryngeal lesion. Tracheotomies are recommended for patients with dyspnea. After their conditions stabilize, decannulation can be successfully performed in most cases. This therapeutic regimen generally delivers a good prognosis.
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PMID:Laryngeal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy): A Retrospective Study of 5 Cases. 2877 Feb 26

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a kind of very rare idiopathic disease. The most common feature is the excessive accumulation of Langerhans cells in lymph nodes, but it may also occur in other areas and lead to related organ damage. We report a case of a 60-year-old man with Rosai-Dorfman disease, which led to dyspnea. Surgery is the best treatment to relieve the patient's dyspnea in a short time. Rosai-Dorfman disease has trend to self-healing, but in the case of special location, surgery should be selected. Some similar mass recurred in the operative area and bilateral nasal cavity in half a year after operation. Now we reviewed the relevant literatures and summarized the experience of diagnosis and treatment in Rosai-Dorfman disease.
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PMID:[A case report of Rosai-Dorfman disease with dyspnea]. 3279 48

Rosai-Dorfman disease (RDD) is a rare disease characterized by dilated lymph node sinuses with a large number of histiocytes. Extranodal disease is considered the uncommon subtype which can be life-threatening when involving vital organs. Here, we report a woman with a 6-month progressive dyspnea who visited our center. Computed tomographic pulmonary angiography revealed a filling defect in her bilateral pulmonary arteries. Bilateral pulmonary artery resection and reconstruction were performed, and postoperative pathology confirmed Rosai-Dorfman disease. To our knowledge, this is the second case of extranodal Rosai-Dorfman disease characterized by invasion of pulmonary artery treated with complete resection.
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PMID:Primary Pulmonary Artery Rosai-Dorfman Disease Treated With Surgical Resection. 3327 43