Title: Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease): A Case Report with Review of Literature
Authors: Dr Rajat Gupta, Dr Rakesh Raina
DOI: http://dx.doi.org/10.18535/jmscr/v4i3.43
Sinus Histiocytosis with Massive lymphadenopathy, also commonly known as Rosai-Dorfman Disease is a rare non neoplastic histiocytic disorder first described by Rosai and Dorfman, seen predominantly in childhood and young adults. It is characterized by fever, painless massive cervical lymphadenopathy and elevated ESR levels. RDD is often misdiagnosed as lymphoma, tuberculosis or metastatic malignancy, so it is important to distinguish it from these conditions as the modes of treatment are different. Here we report a case of 18 years old male presenting with massive lymphadenopathy. Morphological findings demonstrated Emperipolesis consistent with a diagnosis of Rosai-Dorfman disease. Clinical and cytological aspects of the disease are discussed as a rare cause of lymphadenopathy. Keywords: Sinus Histiocytosis with Massive Lymphadenopathy (SHML), Rosai-Dorfman Disease (RDD), Emperipolesis, Lymphadenopathy. 1. Foucar E, Rosai J, Dorfman R: Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease): a review of the entity. Semin Diagn Pathol 1990; 7:19-73. 2. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969; 87(1):63-70. 3. Pulsoni A, Anghel G, Falcucci P, Matera R, Pescarmona E, Ribersani M, et al. Treatment of Sinus histiocytosis with massive lymphadenopathy ( Rosai-Dorfman disease): report of a case and literature review. Am J Hematol 2002; 69(1):67-71. 4. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A pseudolymphomatous benign disorder. Analysis of 34 cases. Cancer 1972; 30:1174-88. 5. Pinto DCG, Vidigal TA, Castro B, Santos BH, DeSousa NJA: Rosai-Dorfman disease in the differential diagnosis of cervical lymphadenopathy. Bras J Otorrinolaringol 2008; 74;632-35. 6. Kumar B, Kraki S, Paudyal P. Diagnosis of Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) by fine needle aspiration cytology. Diagn Cytopathol 2008; 36: 691-95. 7. Shamsian BS, Arabi N, Kazemi A, Rouzrokh M, Ghojehvand N, Azma R, Kaji YM, et al. Rosai-Dorfman Disease: A case report and literature review. Iranian J Blood Cancer 2014; 6(3):155-158. 8. Sodhi KS, Suri S, Nijhawan R, Kang M, Gautam V. Rosai-Dorfman Disease: unusual cause of diffuse and massive retroperitoneal lymphadenopathy. Br J Radiol. 2005; 78(933):845-7. 9. Sharma S, Bhardwaj S, Hans D: Rosai-Dorfman Disease. JK Science 2010; 12(4):194-196. 10. Saleem S, Younas S, Qayyum K: Rosai-Dorfman disease with cervical lymphadenopathy and orbital involvement: A case report. Int J Med Res Health Sci 2015; 4(3):713-716. 11. Khalilzadeh S, Karimi S, Fakharian A, Hakimi SS:Generalized Lymphadeno-pathy: A case report of Rosai-Dorfman Disease. Tanaffos 2007; 6(3):65-67. Abstract
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