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 rare histiocytic proliferative disorder with massive lymphadenopathy. We here describe RDD of a neonate who presented with paleness and hepatosplenomegaly but not lymph-node swelling. Routine laboratory studies showed anemia, thrombocytopenia, and an elevated value of gamma-glutamyl transpeptidase. Histological examination of the liver revealed a proliferation of histiocytes with abundant eosinophilic cytoplasm, which were positive for S-100 protein and CD68 but not CD1a and did not reveal Birbeck granules. Radiological studies showed hepatosplenomegaly and a narrowing of the hepatic vein, which might have contributed to hypersplenism resulting in anemia and thrombocytopenia. This case is thought to be congenital RDD without lymphadenopathy.
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PMID:Congenital Rosai-Dorfman disease without lymphadenopathy. 1469 91

Rosai-Dorfman disease is a rare histiocytic proliferative disorder that commonly presents with a massive lymphadenopathy and a variety of constitutional symptoms. Severe hemolytic anemia is an infrequent complication of this disease. Although the etiology of the condition is unknown, infectious agents including viruses have been implicated. We describe a 2-year-old female child who presented with fever, pallor, and generalized lymphadenopathy complicated by the development of autoimmune hemolytic anemia. A review of the literature of this condition is also provided.
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PMID:Rosai-Dorfman Disease Complicated by Autoimmune Hemolytic Anemia in a Child: A Case Report and Review of the Literature. 2951 11