ÇOKLU ORGAN TUTULUMU OLAN LANGERHANS HÜCRELİ HİSTİOSİTOZİS; OLGU SUNUMU

Langerhans Hücreli Histiositozis (LHH) sebebi tam olarak bilinmeyen nadir görülen bir hastalıktır. Epitel yüzeylerde bulunan, dendritik hücrelerin alt tipi olan monoklonal langerhans hücrelerinin anormal çoğalması ile karakterizedir. Sıklıkla çocuk yaş grubunda gözlenir ancak nadiren erişkin yaş grubunda da gözlenebilmektedir. LHH sıklıkla tek organ tutulumu ile seyreder fakat akciğer, kemik, hipofiz bezi, deri, karaciğer gibi çok sayıda organ tutulumu da gözlenebilir. Organ tutulumlarına göre klinik seyirde farklılıklar ortaya çıkmaktadır. LHH nadir görülen bir hastalık olması ve hastamızda akciğer, karaciğer ve yaygın cilt tutulumu olması nedeniyle sunulmuştur.

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