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)

This study was undertaken to document the grey-scale and power Doppler sonographic features of cervical lymphadenopathy in Kikuchi's disease (histiocytic necrotising lymphadenitis), Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy), Sjogren's syndrome and systemic lupus erythematosus (SLE), which have not been reported in the literature. A retrospective review of the grey-scale and power Doppler sonograms of the cervical lymph nodes in nine patients was conducted (Kikuchi's disease, n = 3; Rosai-Dorfman disease, n = 1; Sjogren's syndrome, n = 1; SLE, n = 4). Lymph nodes were proven to be pathologic by fine-needle aspiration cytology (FNAC). On grey-scale ultrasound (US), lymph nodes were assessed by their distribution, size, shape, echogenicity and internal architecture. The vascular pattern of the lymph nodes was assessed with power Doppler sonography. US features of the lymph nodes were compared to those of metastatic and reactive nodes. In Kikuchi's disease, Rosai-Dorfman disease, Sjogren's syndrome and SLE, the distribution of lymph nodes is similar to that of reactive nodes. Most of the lymph nodes are enlarged with a maximum transverse diameter greater than or equal to 10 mm (83.3 to 100%). In Kikuchi's disease, lymph nodes have grey-scale and Doppler appearances similar to reactive nodes. However, lymph nodes in Rosai-Dorfman disease, Sjogren's syndrome and SLE show similar grey-scale and Doppler features to metastatic nodes. There is no specific US feature to characterise lymphadenopathy from these four miscellaneous causes. Definitive diagnosis should still be based on cytology and histology, and US can help in guiding FNAC for a more accurate cytologic examination.
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PMID:Grey-scale and power Doppler sonography of unusual cervical lymphadenopathy. 1512 Dec 46

Sinus histocytosis with massive lymphadenopathy is a rare disease that has been described by Rosai and Dorfman. It is characterized by massive, cervical lymphadenopathy, with extranodal manifestations in about 40% of patients. It occurs as a distinct entity, never associated with other diseases, and in most cases the prognosis is good. Lymphadenopathy is also a frequent sign of patients with primary Sjogren's syndrome (SS), usually associated with disease activity or concurrent infection. However, excessive lymphadenopathy in SS patients is a sign of lymphoproliferative disorder development. In this report, we describe a patient with primary SS, and excessive lymphadenopathy and splenomegaly who developed Rosai-Dorfman disease, and we discuss the possible aetiopathophysiological mechanism linking these two entities.
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PMID:Sinus histocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with primary Sjogren's syndrome. 1516 14