Rosai-Dorfman Disease
- 1 February 2006
- journal article
- case report
- Published by Ovid Technologies (Wolters Kluwer Health) in Journal of Pediatric Hematology/Oncology
- Vol. 28 (2), 103-106
- https://doi.org/10.1097/01.mph.0000200686.33291.d1
Abstract
Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare and self-limiting benign disorder that most commonly involves the cervical lymph nodes. The authors describe two cases of SHML. Fine-needle aspiration of the lymphadenopathy was performed in both patients. Immunocytochemical and histologic features, as the evidence of emperipolesis and S100 protein positivity on immunostaining, were typical of SHML. Fine-needle aspiration cytology plays an important diagnostic role in SHML and may be conclusive in a typical clinical setting. The diagnosis of SHML should be considered in the differential diagnosis of massive, painless cervical lymphadenopathy. Long-term follow-up is necessary to observe the complete regression of the massive lymphadenopathy. However, specific therapy is available and should be limited to patients with compressive symptoms or extranodal disease.Keywords
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