ROSAİ-DORFMAN HASTALIĞI ( OLGU SUNUMU )

Year 2002, Volume: 16 Issue: 2, 1 - 8, 01.10.2002

Kadri Çırak Tevfik Balıoğlu Serkan Karaca Özlem Keskin Kıraklı Hüseyin Halilçolar Ümit Bayol

Abstract

Sinüs histiyositosiz (Rosai-Dorfman Hastalığı) masif lenfadenopatilerle seyreden, etyolojisi bilinmeyen nadir görülen histiyositik bir bozukluktur. Genellikle çocuk ve adölesanlar etkilenir. Lenfosit ve histiyositlerin lenf nodu sinüslerinde aşırı birikimleriyle meydana gelen masif ağrısız lenfadenopati, ateş, lökositoz, artmış eritrosit sedimentasyon hızı, poliklonal hipergammaglobulinemi ve tipik patolojik bulgularla karekterizedir. Sistemik muayenesinde sol aksiller lenfadenopatisi, standart akciğer radyogramında bilateral hiler dansite artımı, toraks bilgisayarlı tomografisinde bilateral hiler lenfadenopatileri bulunan ve aksiller, skalen bölgelerden alınan eksizyonel biyopsi örneklerinin histopatolojik incelenmesi ile “Rosai-Dorfman Hastalığı “ tanısı alan erkek olguyu sunuyoruz.

Keywords

Rosai-Dorfman hastalığı

ROSAI-DORFMAN DISEASE ( CASE REPORT )

Abstract

Sinüs Histiocytosis With Massive Lymphadenopathy (SHML) (Rosai- Dorfman Disease) is a rare histiocytic disorder of unknown origin. SHML occurs commonly in children and in adolescents and is characterized by painless, massive lymphadenopathy caused by excessive accumulation of lymphocytes and histiocytes distending the lymph node sinuses, fever, leucocytosis, increased erithrocyte sedimentation rate, policlonal hypergammaglobulinemia and typical pathological findings. We present a patient who has a left axillary lymphadenopathy, bilateral hilar dansities on chest X-ray and bilateral hilar lymphadenopathy on thorax computerised tomography and diagnosed as SHML with histopathological examination of exitional lymph node biopsy materials from axillary and scalaneus regions.

Keywords

Rosai-Dorfman disease

References

• 1. Rosai J. Ackerman’s Surgical Patology . St. Louis: Mosby; 1996:1297-8
• 2. Stenberg SS. Diagnostic Surgical Pathology. Philadelphia: Lippincott Williams&Wilkins; 1999: 724-5
• 3. Bekdaş M, Çakmak FN, Karadeniz C, Ergül P, Kapuağası A. Rosai-Dorfman hastalığı (masif lenfadenopatili sinus histiositozisi). Yeni Tıp Dergisi 2000; 17(3):160-1
• 4. Juskevicius R, Finley JL. Rosai-Dorfman disease of the parotid gland: cytologic and histopathologic findings with immunohistochemical correlation. Arch Pathol Lab Med. 2001; 125(10):1348-50
• 5. Bernard F, Sarran N, Serre I, Baldet P, Callamand P, Margueritte G, Astruc J. Sinus Histiocytosis (Destombes-Rosai-Dorfman disease) revealed extranodal spinal involvement. Arch Pediatr. 1999; 6(2):173-7
• 6. Esquivel J, Krishnan J, Jundi M, Sugerbaker PH. Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: first case report. Hepatogastroenterology. 1999; 46(26):1202-5
• 7. Horneff G, Jurgens H, Hort W, Karitzky D, Gobel U. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): response to methotrexate and mercaptopurine. Med Pediatr Oncol 1996; 27(3):187-92
• 8. Carbone A, Passannante A, Gloghini A, Devaney KO, Rinaldo A, Ferlito A. Rewiew of sinus histiocytosis with masive lymphadenopathy (Rosai-Dorfman disease) of head and neck. Ann Otol Rhinol Laryngol 1999; 108(11 Pt 1): 1095- 104
• 9. Das DK, Gulati A, Bhatt NC, Sethi GR. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of two cases with fine needle aspiration cytology. Diag Cytopathol 2001; 24(1):42-5
• 10. Sita G, Guffanti A, Colombi M, Ferrari A, Neri A, Baldini L. Rosai-Dorfman syndrome with extranodal localizations and response to glucocorticoids: a case report. Haematologica 1996; 81:165-7
• 11. Perry BP, Gregg CM, Myers S, Lilly S, Mann KP, Prieto V. Rosai-Dorfman disease (extranodal sinus histiocytosis) in a patient HIV. Ear Nose Throat J. 1998; 77(10):855-8
• 12. Petschner F, Walker UA, Schmitt GA, Uhl M, Peter HH. “Catastrophic systemic lupus erythematosus” with Rosai-Dorfman sinus histiocytosis. succesful treatment with anti-CD 20/rutuximab. Dtsch Med Wochenschr 2001; 126( 37):998-1001
• 13. Hicke A, Wieczorek M, Olejnik I. Rosai-Dorfman syndrome in a 17 year old boy as a diagnostic problem of lympadenopathy. Acta Haematol Pol 1995; 26(4):425-8