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sinus histiocytosis with massive lymphadenopathy

Rosai-Dorfman disease, Destombes disease

 
Sinus histiocytosis with massive lymphadenopathy is an idiopathic proliferation of unique histiocytes that have vesicular nuclei and voluminous pale cytoplasm, often with emperipolesis.

Associations

-  Langerhans histiocytosis
-  autoimmune lymphoproliferative syndrome (ALPS) (#15958855#)

Localization

-  lymph nodes
-  nasal fossae (#8958550#)
-  retro-orbital space (#8958550#)
-  skin

Immunohistochemistry

-  CD68 +
-  S-100 protein +
-  CD1a -



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