Home > D. Systemic pathology > Hematopathology > Rosai-Dorfman disease

Rosai-Dorfman disease

Tuesday 16 December 2003

Ent. 1969.

Digital cases

- HPC:290

Localization

- lymph node (nodal Rosai-Dorfman disease)
- extranodal Rosai-Dorfman disease

  • skin (#12558621#), hypodermis (#1498408#, #3401030#)
  • pleura (#14652824#)
  • intracranial (#14608579#, #12637307#), brain (#12601463#), meninges
  • nasal region (#12761707#)
  • breast (#12760958#, #9416494#, #9199644#)
  • thyroid (#12699598#, #12683901#)
  • bone (#12652340#, #9653919#, #9526780#, #2013464#)
  • gastrointestinal tract (#12562257# #10746683#, #17400399#)
  • eye (#12479625#), lacrymal gland
  • kidneys (#10587134#, #10471758#, #9072605#, #1456886#)
  • pancreas (#10370692#, #11555773#)
  • salivary glands (#9472588#)
  • liver (#8771474#)
  • larynx
  • heart (cardiac Rosai-Dorfman disease) (#19962176#)

Types

- mostly isolated
- rarely multicentric (#12622452#)

Synopsis

- large, pale, histiocytic cells (Rosai-Dorfman cells) exhibiting emperipolesis (cytophagocytosis)

Immunohistochemistry

- S-100+
- CD68+
- CD1a-

Electron microscopy

- Birbeck granules are absent

Associations

- acute lymphoblastic leukemia (##11496358#)
- lymphoma (#9031615#, #9006752#)
- follicular lymphoma (#10786808#)
- nodular lymphocyte predominant Hodgkin disease (#10786808#, #8522313#)
- chronic autoimmune thyroiditis (#10710276#)
- inflammatory pseudotumor (#9059366#)
- systemic lupus erythematosus (#11555773#)

References