Rosai-Dorfman Disease



Rosai-Dorfman Disease


Rose Anton, MD

John M. Stewart, MD, PhD





Key Facts


Clinical Issues



  • Usually asymptomatic adenopathy in young patients


  • Spontaneous regression occurs in most patients


  • No specific therapy is required


Cytopathology



  • RDD histiocytes



    • Large size with abundant eosinophilic cytoplasm


    • Defined cell border


    • Emperipolesis is usual


  • Round vesicular nucleus



    • Distinct central nucleolus


Ancillary Tests



  • Immunohistochemistry



    • S100(+), CD1a(-)


Top Differential Diagnoses



  • Langerhans cell histiocytosis


  • Chronic granulomatous inflammation


  • Kikuchi lymphadenitis







(A) Emperipolesis image is subtle in this Diff-Quik-stained smear and can easily be dismissed as just tingible body macrophages due to inflammation/repair. (B) Diff-Quik-stained smear shows an RDD histiocyte containing lymphocytes image and sparse tingible debris image. (C) Pap-stained smear shows RDD histiocytes with variable numbers of lymphocytes. The eye is quickly drawn to cells with significant emperipolesis image. (D) Pap-stained smear shows a high-power view of emperipolesis. Note that the lymphocytes image and plasma cells image appear viable and enclosed in a defined space within the cytoplasm of the RDD histiocyte.

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Jul 6, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Rosai-Dorfman Disease

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