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Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease)

View larger version (139K): [in this window] [in a new window]   Figure 1. This low power magnification of a lymph node stained with H&E shows marked expansion of the sinuses and thickening of the capsule.

View larger version (154K): [in this window] [in a new window]   Figure 2. This medium power magnification (100X) reveals that sinuses are expanded by histiocytes with abundant cytoplasm

View larger version (129K): [in this window] [in a new window]   Figure 3. High power magnification (immersion oil 1000 X) reveals histiocytes, with abundant cytoplasm and vesicular nuclei, engulfing many lymphocytes, a process known as emperipolesis.

	Clinical Summary

TOP Clinical Summary Diagnosis Discussion Differential Diagnosis

Sex
Male

Age
29

Ethnicity
Caucasian

	Diagnosis

TOP Clinical Summary Diagnosis Discussion Differential Diagnosis

 
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease)

	Discussion

TOP Clinical Summary Diagnosis Discussion Differential Diagnosis

 
This case is a typical of sinus histiocytosis with massive lymphadenopathy.  The majority of patients are young, with a slight male predominance. Lymph nodes are the most common site of involvement ;however, rare cases of other extranodal sites, including upper respiratCory system, gastrointestinal system, orbit, and meningies, have been reported. The disease is usually self-limited and requires no treatment ;however, rarely, it may persist and even cause death.
The characteristic histology as shown here is the expansion of the sinuses with large histiocytes revealing emperipolesis (Figures1, 2, 3). Finding histiocytes demonstrating engulfed lymphocytes is the most crucial aspect of the correct diagnosis.

	Differential Diagnosis

TOP Clinical Summary Diagnosis Discussion Differential Diagnosis

 
Reactive lymphoid hyperplasia with sinus histiocytosis is the main differential diagnosis. Anaplastic large cell lymphoma and Hodgkin lymphoma should also be considered in the differential diagnosis.

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This Article Figures Only Services Similar articles in this journal Alert me to new content in the Image Bank Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Alkan, S. Search for Related Content PubMed Articles by Alkan, S. Related Collections Reactive Conditions of Lymph Nodes and Spleen Related ASH-SAP Chapter Related Image Bank Image Sets Related ASH Education Book Article Social Bookmarking                   What's this?