Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Evidence for a dendritic cell derivation?

Abul Ala Syed Rifat Mannan, Asis K. Karak

Research output: Contribution to journalArticlepeer-review

7 Scopus citations


Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease is a rare histiocytic disorder of unknown origin. Both dendritic cell and monocyte-macrophage lineage have been considered as the origin of the histiocytes that are classical of the disorder. We analyzed seven cases of Rosai-Dorfman disease to determine the immunophenotypic profile of these cells. Immunohistochemical analysis was undertaken by using: a) fascin as a novel mature dendritic cell marker; b) S-100 protein; c) CD68 (both KP1 and PGM1) as macrophage related markers; d) CD79a and e) CD3. The Rosai-Dorfman histiocytes exhibited strong cytoplasmic staining for fascin in all the cases and demonstrated formation of a meshwork affine dendritic processes emanating from the cell cytoplasm. S-100 protein was uniformly expressed in all the cases. Expression of both KP1 and PGM1 epitopes of CD68 was found to be weak and variable. The phagocytosed lymphocytes were composed of an admixture of both B and T-lymphocytes. The characteristic expression pattern of the histiocytes for fascin along with co-expression of S-100 protein and a consistently weaker and variable expression ofmacrophage-lineage markers point more towards a dendritic cell ontogeny of these cells. Further, abundant fascin expression and presence of dendritic processes indicate a differentiated or mature dendritic cell phenotypefor these cells.

Original languageEnglish (US)
Pages (from-to)300-304
Number of pages5
JournalIndian Journal of Pathology and Microbiology
Issue number3
StatePublished - Jul 1 2005


  • Dendritic cell
  • Fascin
  • Rosai-Dorfman disease
  • SHML

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Microbiology (medical)


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