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 (RDD) is a proliferative histiocytic disorder of unknown etiology, which is characterized by sinus histiocytosis with massive lymphadenopathy (1). In most cases, RDD has a benign course and treatment is not necessary. However, severe cases of RDD require treatment, and the treatment strategy is determined on the basis of the severity of the disease or the extranodal involvement of vital organs. We report a single case of RDD with atypical presentation of persistent constitutional symptoms, progressing pathologic fractures, and end-organ dysfunction, including acute kidney failure and liver cirrhosis with esophageal varices.
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PMID:A single case of rosai-dorfman disease marked by pathologic fractures, kidney failure, and liver cirrhosis treated with single-agent cladribine. 2540 Oct 88

Patients with cirrhosis of the liver usually present with a small shrunken liver and a large spleen. The presence of an unusually huge liver should prompt the treating doctor to look for another cause, as this may be treatable and improve the patient's outcome. In South Africa tuberculosis and lymphoma in the presence of HIV infection should be excluded. Sinus histiocytosis with massive lymphadenopathy and cirrhosis is a rare combination and has not been reported before in the literature. This case is intended to make clinicians aware of this rare combination of diseases.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and cirrhosis of the liver: A case report and literature review. 2713 63