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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