Rosai-Dorfman Disease: An Imposter of Plasma Cell Rich Diseases
Keywords:
Rosai Dorfmann Disease, Generalised lymphadenopathy, Plasma cell
Abstract
Rosai Dorfman Disease is a histiocytic disorder associated with painless cervical lymphadenopathy. The typical histomorhology includes dilated lymphatic sinuses with a lymphohistiocytic infiltrate with emperipolesis of lymphoid cells and plasma cells as the hallmark feature. We are highlighting a unique case of Rosai Dorfman disease presenting with generalized progressive lymphadenopathy in a 19 year old male, in whom diagnosis of RDD was established by fine needle aspiration cytology. The case that is discussed here is also worth documenting because of its unusual morphology which is dominated by plasma cells. We have also attempted to determine a possible association of this case of Rosai Dorfman disease with increased IgG4+ plasma cells and IgG4 related disease.References
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2. Menon MP, Evbuomwan MO, Rosai J, Jaffe ES, Pittaluga S. A subset of Rosai-Dorfman disease cases show increased IgG4-positive plasma cells: another red herring or a true association with IgG4-related disease? Histopathology. 2014 Feb;64(3):455–9.
3. Zhang X, Hyjek E, Vardiman J. A subset of Rosai-Dorfman disease exhibits features of IgG4-related disease. Am J Clin Pathol. 2013 May;139(5):622–32.
4. Garces S, Medeiros LJ, Patel KP, Li S, Pina-Oviedo S, Li J, et al. Mutually exclusive recurrent KRAS and MAP2K1 mutations in Rosai-Dorfman disease. Mod Pathol Off J U S Can Acad Pathol Inc. 2017 Oct;30(10):1367–77.
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6. Ju J, Kwon YS, Jo KJ, Chae DR, Lim JH, Ban HJ, et al. Sinus Histiocytosis with Massive Lymphadenopathy: A Case Report with Pleural Effusion and Cervical Lymphadenopathy. J Korean Med Sci. 2009 Aug;24(4):760–2.
7. Kumar B, Karki S, Paudyal P. Diagnosis of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) by fine needle aspiration cytology. Diagn Cytopathol. 2008 Oct;36(10):691–5.
8. Vemuganti GK, Naik MN, Honavar SG. Rosai dorfman disease of the orbit. J Hematol OncolJ Hematol Oncol. 2008 Jun 28;1:7.
9. Kala C, Agarwal A, Kala S. Extranodal manifestation of Rosai-Dorfman disease with bilateral ocular involvement. J Cytol. 2011 Jul;28(3):131–3.
10. Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR, Velazquez-Vega J, Borja MJ, Rosenberg S, et al. Rosai-Dorfman disease of the central nervous system: report of 6 cases and review of the literature. Medicine (Baltimore). 2014 May;93(3):165–75.
11. Bist SS, Bisht M, Varshney S, Pathak VP. Rosai Dorfman syndrome with extranodal manifestation. J Assoc Physicians India. 2007 Jun;55:445–7.
12. Khalilzadeh S, Karimi S, Fakharian A, Hakimi SS. Generalized lymphadenopathy: A case report of Rosai-Dorfman disease. Tanaffos. 2007;6(3):65–7.
13. Emile J-F, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016 02;127(22):2672–81.
14. Garza-Guajardo R, García-Labastida LE, Rodríguez-Sánchez IP, Gómez-Macías GS, Delgado-Enciso I, Chaparro MMS, et al. Cytological diagnosis of Rosai-Dorfman disease: A case report and revision of the literature. Biomed Rep. 2017 Jan;6(1):27–31.
2. Menon MP, Evbuomwan MO, Rosai J, Jaffe ES, Pittaluga S. A subset of Rosai-Dorfman disease cases show increased IgG4-positive plasma cells: another red herring or a true association with IgG4-related disease? Histopathology. 2014 Feb;64(3):455–9.
3. Zhang X, Hyjek E, Vardiman J. A subset of Rosai-Dorfman disease exhibits features of IgG4-related disease. Am J Clin Pathol. 2013 May;139(5):622–32.
4. Garces S, Medeiros LJ, Patel KP, Li S, Pina-Oviedo S, Li J, et al. Mutually exclusive recurrent KRAS and MAP2K1 mutations in Rosai-Dorfman disease. Mod Pathol Off J U S Can Acad Pathol Inc. 2017 Oct;30(10):1367–77.
5. Sennes L, Koishi H, Cahali R, Sperandio F, Butugan O. Rosai-Dorfman disease with extranodal manifestation in the head. Ear Nose Throat J. 2004 Dec;83(12):844–7.
6. Ju J, Kwon YS, Jo KJ, Chae DR, Lim JH, Ban HJ, et al. Sinus Histiocytosis with Massive Lymphadenopathy: A Case Report with Pleural Effusion and Cervical Lymphadenopathy. J Korean Med Sci. 2009 Aug;24(4):760–2.
7. Kumar B, Karki S, Paudyal P. Diagnosis of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) by fine needle aspiration cytology. Diagn Cytopathol. 2008 Oct;36(10):691–5.
8. Vemuganti GK, Naik MN, Honavar SG. Rosai dorfman disease of the orbit. J Hematol OncolJ Hematol Oncol. 2008 Jun 28;1:7.
9. Kala C, Agarwal A, Kala S. Extranodal manifestation of Rosai-Dorfman disease with bilateral ocular involvement. J Cytol. 2011 Jul;28(3):131–3.
10. Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR, Velazquez-Vega J, Borja MJ, Rosenberg S, et al. Rosai-Dorfman disease of the central nervous system: report of 6 cases and review of the literature. Medicine (Baltimore). 2014 May;93(3):165–75.
11. Bist SS, Bisht M, Varshney S, Pathak VP. Rosai Dorfman syndrome with extranodal manifestation. J Assoc Physicians India. 2007 Jun;55:445–7.
12. Khalilzadeh S, Karimi S, Fakharian A, Hakimi SS. Generalized lymphadenopathy: A case report of Rosai-Dorfman disease. Tanaffos. 2007;6(3):65–7.
13. Emile J-F, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016 02;127(22):2672–81.
14. Garza-Guajardo R, García-Labastida LE, Rodríguez-Sánchez IP, Gómez-Macías GS, Delgado-Enciso I, Chaparro MMS, et al. Cytological diagnosis of Rosai-Dorfman disease: A case report and revision of the literature. Biomed Rep. 2017 Jan;6(1):27–31.
Published
2018-12-27
Issue
Section
Case Report
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