Çocukluk Çağında Ender Görülen Bir Lenfadenopati Nedeni: Masif Lenfadenopatili Sinüs Histiositozu RosaiDorfman Hastalığı

Abstract

Sinüs histiyositosizi Rosai-Dorfman Hastalığı , ender göz- lenen histiyositik bir hastalık olup, etiyolojisi tam olarak bilinmemektedir. Lenfosit ve histiyositlerin lenf nodu sinüs- lerinde birikmesiyle oluşan masif lenfadenopatiler, hastalı- ğın en karakteristik özelliğidir. Servikal lenfatikler en sık tutulan bölge olmasına rağmen, diğer lenf nodlarında da tutulum görülebilir. Tanı patolojik olarak konur. Tedavisiz izlenen semptomsuz vakaların yanında, yaşamı tehdit edici organ tutulumları nedeniyle immunsupresif tedavi, cerrahi ve radyoterapi uygulanan vakalar da mevcuttur. Boyun bölgesinde masif lenfadenomegalileri nedeniyle, ayırıcı tanıda malin hastalık düşünüldüğü için polikliniğimize gönderilen ve eksizyonel biyopsi örneklerinin histopatolo- jik incelenmesi ile “Rosai-Dorfman Hastalığı”tanısı alan iki vaka literatür eşliğinde sunulmuştur

Keywords

• Lenfadenomegali,çocuk,RosaiDorfman

A Rare Cause of Childhood Lymphanedopathy: Sinus Histiocytoes with Massive Lymphadenopathies

Abstract

The sinus histiocytosis Rosai-Dorfman Disease is a rarely seen histiocytic disease and its etiology is not clearly known. Massive lymphadenopathies that are formed by accumulation of lymphocytes and histiocytes in lymph node sinuses are the main characteristics of the disease. Although cervical lymphatics are the most frequently affected region , the other lymph nodes can also be involved. The diagnosis is histopathologically established. In addition to asympto- matic cases followed up without any treatment, there are also cases requiring immunosuppressive therapy, surgical treatment or radiotherapy due to life-threatening organ involvement. Hereby, we present two cases diagnosed with Rosai-Dorfman Disease on histopathological examination. In these cases, the malignancies were considered in diffe- rential diagnosis because of massive lymphadenopathies in cervical area

Keywords

• Lymphadenopathy,child,Rosai-Dorfman

References

1. 1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969;87:63-70.
2. 2. Kliegman RB, ed. Richard L. Tower II, Bruce M. Camitta. Nelson Textbook of Pediatrics 20th -Chapter 484- Sinus Histiocytosis with Massive Lymphadenopathy (RosaiDorfman Disease) 484.2
3. 3. Gedikoğlu G, Koc L, Devecioğlu O, Celik A, Ağan M, Salman T. Masif lenfadenopatili sinüs histiyositozu. Proceedings of 6. Turkish National Cancer Congress 1985.
4. 4. Scumpia AJ, Frederic JA, Cohen AJ, Bania M, Hameed A, Xiao PQ. Isolated intracranial Rosai-Dorfman disease with orbital extension. J Clin Neurosci 2009;16:1108-9. http://dx.doi.org/10.1016/j.jocn.2008.10.022
5. 5. Lussier C, Klijanienko J, Brisse H, Quintana E, Vielh P, Brousse N. Cytology of Rosai-Dorfman disease. Diagn Cytopathol 2001;24:298-300. http://dx.doi.org/10.1002/dc.1064
6. 6. Gupta P, Babyn P. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfmandisease): a clinicoradiological profile of three cases including two with skeletal disease. Pediatr Radiol 2008;38:721-8. http://dx.doi.org/10.1007/s00247-007-0701-0
7. 7. Wu M, Anderson AE, Kahn LB. A report of intracranial Rosai-Dorfman disease with literature review. Ann Diagn Pathol 2001;5:96-102. http://dx.doi.org/10.1053/adpa.2001.23027
8. 8. Gupta K, Bagdi N, Sunitha P, Ghosal N. Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child: a case report and review of the literature. Br J Radiol 2011;84(1003):e138-41. http://dx.doi.org/10.1259/bjr/15772106

9. 9. Noguchi S, Yatera K, Shimajiri S, Inoue N, Nagata S, Nishida C, et al. Intrathoracic Rosai-Dorfman disease with spontaneous remission: a clinical reportand a review of the literature. Tohoku J Exp Med 2012;227:231-325. http://dx.doi.org/10.1620/tjem.227.231
10. 10. Chang YC, Tsai MH, Chen CL, Tsai CH, Lee AY. Nasal Rosai-Dorfman disease with intracranial involvement: a case report. Am J Otolaryngol 2003;24:183-6. http://dx.doi.org/10.1016/S0196-0709(03)00002-4