ROSAİ-DORFMAN HASTALIĞI ( OLGU SUNUMU )

Öz

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.

Anahtar Kelimeler

• Rosai-Dorfman hastalığı

ROSAI-DORFMAN DISEASE ( CASE REPORT )

Öz

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.

Anahtar Kelimeler

• Rosai-Dorfman disease

Kaynakça

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