Sinus histiocytosis with massive lymphadenopathy: evidence for its relationship to macrophages and for a cytokine-related disorder

Histopathology. 1999 Dec;35(6):525-33. doi: 10.1046/j.1365-2559.1999.00746.x.


Aims: Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease is a rare histiocytic disorder of unknown origin. Immunophenotypically the histiocytes of SHML express intensively the S100 protein and in addition a panel of macrophage-associated antigens. Their exact relationship to either monocytes/macrophages or immune accessory dendritic cells is, however, still controversial.

Methods and results: In this report recurrent nodal and extranodal manifestations of SHML of a 70-year-old patient were analysed by differential phenotyping using a panel of monoclonal and polyclonal antibodies to macrophage and immune accessory dendritic cell related antigens and by applying nonradioactive in-situ hybridization.

Conclusions: We conclude that stimulation of monocytes/macrophages via macrophage colony stimulating factor (M-CSF) leading to immune suppressive macrophages represents a main mechanism for the pathogenesis of SHML. The study further provides evidence for the monocyte/macrophage but not dendritic cell differentiation of SHML histiocytes.

Publication types

  • Case Reports

MeSH terms

  • Biomarkers / analysis
  • Dendritic Cells / metabolism
  • Dendritic Cells / pathology
  • Histiocytes / metabolism
  • Histiocytes / pathology*
  • Histiocytosis, Sinus / etiology
  • Histiocytosis, Sinus / metabolism
  • Histiocytosis, Sinus / pathology*
  • Humans
  • Immunophenotyping
  • In Situ Hybridization
  • Lymph Nodes / metabolism
  • Lymph Nodes / pathology*
  • Macrophage Colony-Stimulating Factor / metabolism*
  • Macrophages / metabolism
  • Macrophages / pathology*
  • Male
  • Middle Aged
  • Monocytes / metabolism
  • Monocytes / pathology


  • Biomarkers
  • Macrophage Colony-Stimulating Factor