Langerhans hücreli histiositoz, dendritik hücre sistemine ait hücrelerin lokalize yada yaygin proliferasyonu ile karekterize bebek ve küçük çocuklarda görülen nadir bir hastaliktir.Hastalarin çogunda belirtilerin ortaya çikisi ile tani arasindaki süre 1-3 aydir.Burada ilk belitilerin ortaya çikisi ile tani arasinda 14 ay gecikme olan basvuruda yaygin kasintili cilt lezyonlari, sol kulakta pürülan otit ,saçli deride yumusak doku sislikleri, propitozis,üst dislerde sallanma, hepatosplenomegali, anemi,çok su içme,çok idrar yapma gibi diabetes insipitus tablosu olan 2.5 yasinda bir vaka sunuldu.Cilt ve kemik iligi biyopsisi ile langerhans hücreli hisiositoz tanisi alan hasta sistemik kemoterapiye iyi yanit verdi. Kasintili cilt lezyonlari nedeniyle defalarca doktora basvuran, egzema tanisiyla önerilen topikal tedavilerden yarar saglanamayan ,taninin gecikmesi nedeniyle çok sayida organ tutulumu gelisen vaka klinigi ve direkt grafileriyle tipik olmasi açisindan sunuldu.
Tanida Gecikme Nedeniyle Yaygin Organ Tutulumu Gözlenen Bir Langerhans Hücreli Histiositoz - POSTER: 57
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Tanida Gecikme Nedeniyle Yaygin Organ Tutulumu Gözlenen Bir Langerhans Hücreli Histiositoz - POSTER: 57
J Curr Pediatr 2005;3(1)
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- 1Ssk Ankara Çocuk Hastaliklari Egitim Hastanesi, Ankara
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