Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare, benign, non-Langerhanscell, histiocytic proliferative disease without any known cause.1 Histopathological hallmark of Rosai-Dorfman disease is characteristic histiocytes with abundant pale cytoplasm exhibiting emperipolesis.
Physical examination revealed fever of 39AdegC, she had tender, mobile bilateral cervical lymphadenopathy. Moreover she also had positive fluid thrill and mild pedal edema.
The medical records of 56 children with lymphadenopathy between 2014 and 2017 were reviewed retrospectively at Ege University, Children's Hospital, General Pediatrics Unit.
During April 2009-May 2014, eighty immunocompetent patients (73 children, 7 adults) in Japan who were suspected of having CSD because of fever with or without lymphadenopathy, and a history of contact with cats or dogs were referred to us for serologic and molecular diagnosis of CSD.
The patient's most recent staging imaging after completion of chemotherapy revealed excellent response with resolution of retroperitoneal and pelvic lymphadenopathy with decreased enhancement of multiple bone lesions (Figures 2(c) and 2(d)).
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