Zeynep BAYRAMOĞLU, Muhammet Zeki GÜLTEKİN, Yaşar ÜNLÜ

Erişkin Başlangıçlı Langerhans Hücreli Histiyositoz

Langerhans hücreli histiyositoz (LHH), Langerhans hücrelerinin klonal ve neoplastik proliferasyonu ile karakterize nadir bir hastalıktır. En sık çocuklarda tanı almakla beraber, her yaşta görülebilir. Klinik bulgular, tutulan organ sistemine göre değişmektedir. LHH'nin kesin tanısı histopatolojik olarak konulmaktadır. LHH’un tedavisi, hastalığın bulunduğu organ sistemine ve lezyonların yaygınlığına göre planlanmaktadır. Biz burada erişkin yaş başlangıçlı, femur tutulumu olan 33 yaşında erkek hastanın klinik, radyolojik ve histapatolojik bulguları sunularak güncel literatür eşliğinde tartışmayı amaçladık.

Anahtar Kelimeler:

histiyositozis, erişkin başlangıç, Langerhans hücreleri, erişkin başlangıç

Adult Onset Langerhans Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is a rare disease characterized by clonal and neoplastic proliferation of Langerhans cells. It is most commonly seen in children but can be seen at any age. Clinical findings depend on the organ system involved. The diagnosis of LCH is made histopathologically. The treatment is planned according to the organ system of the disease, the prevalence of the lesions and the presence of specific organ dysfunction. We aimed to discuss the clinical, radiological and histapatological findings of a 33-year-old man with femoral involvement at adulthood and discuss the current literature.

Keywords:

Langerhans cells, histiocytosis, adult onset,

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