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osseous Rosai-Dorfman disease

Sunday 28 January 2007, by JA

Definition : Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder of unknown etiology.

Most patients present with lymph node involvement manifesting as adenopathy; however, RDD may arise primarily in a variety of extranodal sites, including bone.

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a rare nonneoplastic disorder of unknown etiology characterized by massive, painless bilateral neck lymph node enlargement. Bone involvement is extremely uncommon.

The classic microscopic picture is that of large histiocytes containing lymphocytes within cytoplasmic vacuoles (emperipolesis and lymphocytophagocytosis). Some histiocytes may exhibit atypia and multinucleation.

Polyclonal plasma cells with Russell bodies may also be seen. The histiocytes are positive for S100, CD68, CD30, and oil red O stains, whereas CD1a is negative.

Differential diagnosis

In contrast to Rosai-Dorfman disease, xanthogranuloma lacks emperipolesis, and the histiocytes do not express S100.

See also

- Rosai-Dorfman disease

References

- Primary Rosai-Dorfman Disease of Bone: A Clinicopathologic Study of 15 Cases. Demicco EG, Rosenberg AE, Bj√∂rnsson J, Rybak LD, Unni KK, Nielsen GP. Am J Surg Pathol. 2010 Sep;34(9):1324-33. PMID: 20679880

- Extranodal Rosai-Dorfman disease with multifocal bone and epidural involvement causing recurrent spinal cord compression. Al-Saad K, Thorner P, Ngan BY, Gerstle JT, Kulkarni AV, Babyn P, Grant RM, Read S, Laxer RM, Chan HS. Pediatr Dev Pathol. 2005 Sep-Oct;8(5):593-8. PMID: 16211443