Rosai-Dorfman Disease

Related Cases: HP-0314

Clinical Futures

  • AKA: Sinus histiocytosis with massive lymphadenopathy
  • Mostly young patients, slightly male performance
  • Commonly involves lymph nodes, especially cervical nodes; 25-40% extranodal
  • Presentations: Painless, massive bilateral cervical lymphadenopathy; fever, leukocytosis, polyclonal gammopathy
  • Prognosis: Excellent in most patients with spontaneous regression of lymph nodes

Microscopic Findings

  • Preserved but often distorted architecture with marked sinus expansion 

  • Thickened and fibrotic lymph node capsule in most cases

  • RDD histiocytes in sinuses:  

    • Large size with mild cytologic atypia; no necrosis or atypical mitosis 

    • Round nuclei, open chromatin, often prominent nucleoli 

    • Abundant pale or pink cytoplasm

    • Emperipolesis: Viable lymphocytes, plasma cells and neutrophils in cytoplasmic vacuoles 

  • Atrophic cortex and sinus cords with plasmacytosis

  • Can be seen focally in Hodgkin or non-Hodgkin lymphomas, incidental findings with no clinical impact

Immunohistochemistry Staining

  • Histiocytes S100+, CD68+, CD1a-, langerin-

  • May have increased IgG4+ plasma cells

Differential Diagnosis

  • Reactive Sinus Histiocytosis

    • Lack of significant expansion of sinuses

    • Smaller histiocytes with less cytoplasm and no or rare emperipolesis

    • Histiocytes S100-, CD1a-

  • Langerhans Cell Histiocytosis

    • May have sinusoidal pattern in early stage of disease

    • Epithelioid cells with nuclear grooves, atypical mitoses and necrosis

    • Lack of emperipolesis

    • Abundant reactive eosinophils

    • CD1a+, S100+, langerin+

  • Extranodal IgG4-Related Disease

  • Prominent fibrosis, plasmacytosis and increase in IgG4+ cells

  • Distinction is made based on overall clinical and histologic features



Last update: 10/06/2017