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digestive Rosai-Dorfman disease

Tuesday 20 August 2019

Rosai-Dorfman Disease of the Digestive System

Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytic proliferation that occurs in nodal and extranodal sites. Rare examples of the disease involving the digestive system have been described.

The lesions were generally composed of polygonal to spindle-shaped histiocytes with eosinophilic to clear cytoplasm admixed with lymphoplasmacytic cells.

The inflammatory cells formed lymphoid aggregates and included focally scattered or small collections of neutrophils in some cases.

Fibrosis was variable, and some cases had a storiform pattern.

Vasculopathy in the form of a thickened capillary wall, medium-sized arterial wall infiltration by lesional and inflammatory cells and phlebitis can be seen. All cases were reactive for S100-protein.

Paywall references

- Rosai-Dorfman Disease of the Digestive System-Beware Vasculopathy: A Clinicopathologic Analysis. Alruwaii ZI, Zhang Y, Larman T, Miller JA, Montgomery EA. Am J Surg Pathol. 2019 Aug 12. doi : 10.1097/PAS.0000000000001343 PMID: 31414989