Rosai-Dorfman disease with spontaneous resolution: case report of a child

Rev Bras Hematol Hemoter. 2011;33(4):312-4. doi: 10.5581/1516-8484.20110083.


Rosai-Dorfman disease is a self-limiting condition caused by histiocyte proliferation within the sinusoids of lymph nodes and in extranodal tissue. It is a rare disease, particularly in children, that progresses with extensive lymphadenopathy. This paper reports on the case of a 2-year-old child with progressive cervical lymphadenopathy associated with persistent fever and radiological findings suggestive of lymphoma. Histopathological and immunohistochemistry studies of a lymph node biopsy established the diagnosis of Rosai-Dorfman disease. Both lymphadenopathy and fever resolved spontaneously.

Keywords: Child; Histiocytosis, non-Langerhans-cell; Histiocytosis, sinus; Humans; Lymph nodes; Lymphatic diseases.