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Rosai-Dorfman Disease

Keywords: Sinus histiocytosis with massive lymphadenopathy (SHML) , Lymphophagocytosis. Emperipolesis , Rosai-

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Abstract:

Rosai-Dorfman Disease (RDD) or Sinus Histiocytosis with massive lymhadenopathy (SHML) is a rare,benign proliferative disorder of histiocytes, sometimes showing familial incidence. It is mainly characterizedby painless bilateral cervical lymph node enlargement and is often associated with fever and leucocytosis.It presents in the first or second decades of life, though any age group can be affected. Without theawareness about RDD, the diagnosis of RDD is unexpected especially in South East Asian Countrieswhere certain lymphadenopathies such as tuberculosis, metastatic nasopharayngeal carcinoma andlymphomas are common. RDD is commonly missed and so its revision would seem worthwhile, this caseillustrating the disappearance of the lymphadenopathy without treatment.

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