Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
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 originally defined as a relatively specific benign pseudolymphomatous disorder. Although the etiology remains unknown, the spectrum of SHML has been expanded to include predominance of extranodal disease in some patients, clinically significant immunologic abnormalities in 10% of patients, and fatal outcome in 7% of patients. We report the rare occurrence of SHML in identical twins; to our knowledge, SHML in identical twins has been reported only once previously. The two patients described are also unusual because of the predominance of retroperitoneal disease with minimal peripheral adenopathy. After a seven-year clinical course, one twin died of extensive retroperitoneal disease, liver failure, bleeding diathesis, and seizure disorder. The other twin is alive after a six-year course of progressive retroperitoneal disease.
 ...
PMID:Sinus histiocytosis with massive lymphadenopathy. Occurrence in identical twins with retroperitoneal disease. 334 28

A 56-year-old woman with vertex headaches, new-onset seizure disorder, nasal obstruction, and hyposmia was found to have a dural-based intracranial mass as well as bilateral masses arising from the inferior turbinates and extending into the nasopharynx. The patient underwent endoscopic resection of the involved portions of the inferior turbinates, including the sizable nasopharyngeal component. Craniotomy was performed at a later date. Both pathologic specimens were consistent with extranodal Rosai-Dorfman disease. The patient showed resolution of all symptoms after surgery. Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is a rare entity usually presenting as cervical nodal disease. When extranodal disease of the head and neck is present, prompt recognition and conservative surgical management is the treatment of choice for this benign pseudolymphomatous entity.
 ...
PMID:Rosai Dorfman disease presenting as synchronous nasal and intracranial masses. 1460 79