Oksipital kemik yerleşimli langerhans hücreli histiyositoz: Olgu sunumu

Langerhans Cell Histiocytosis is termed as a group of diseases that leads to have same histopathology, but different clinical symptoms. The disease has 3 distinct forms and the localized form is termed as Eosinophilic granuloma . Eosinophilic granuloma is the localized form of which has the least clinical severity and the most favourable subtype in Langerhans Cell Histiocytosis group. The treatment options are available as surgery removal, chemotherapy, and radiotherapy alone or in combinations. In this paper, a young adult patient was represented who referred to our clinic with complaints of swelling and pain on right occipital region, and who diagnosed as Langerhans Cell Histiocytosis (Eosinophilic granuloma) after evaluation of radiologic tests and histopathologic survey.

Özet

Langerhans Hücreli Histiyositozis aynı histopatolojiye sahip fakat farklı klinik semptomlara neden olan hastalıklar grubu olarak adlandırılmaktadır. Üç ayrı formu olan hastalığın lokalize formu Eozinofilik granulom olarak isimlendirilmektedir. Eosinofilik granülom, Langerhans Hücreli Histiyositozis grubu içerisinde klinik seyri en az şiddetli ve lokalize form olup en iyi prognoza sahip alt tiptir. Tedavi şeçenekleri arasında, cerrahi eksizyon, kemoterapi, radyoterapi tek başına kullanılabileceği gibi kombine kullanımları da bulunmaktadır. Burada, sağ oksipital bölgede şişlik ve ağrı şikayeti ile kliniğimize başvuran, radyolojik tetkikleri ve histopatolojik incelemesi sonucunda Langerhans Hücreli Histiyositozis (Eosinifilik granülom) tanısı alan genç erişkin bir hasta sunuldu.

Anahtar sözcükler: Langerhans hücreli histiyositozis, eozinofilik granülom, soliter kemik lezyonu

 

Abstract

Langerhans Cell Histiocytosis is termed as a group of diseases that leads to have same histopathology, but different clinical symptoms. The disease has 3 distinct forms named as fulminant (Letterer-Siwe disease), chronic recurrent (Hand-Schüller-Christian disease) and localized (Eosinophilic granuloma). Eosinophilic granuloma is typically found in children and is characterized with one or more lytic bone lesions.  Eosinophilic granuloma is the localized form of which has the least clinical severity and the most favourable subtype in Langerhans Cell Histiocytosis group. The treatment options are available as surgery removal, chemotherapy, and radiotherapy alone or in combinations. In this paper, a young adult patient was represented who referred to our clinic with complaints of swelling and pain on right occipital region, and who diagnosed as Langerhans Cell Histiocytosis (Eosinophilic granuloma) after evaluation of radiologic tests and histopathologic survey. 

Keywords: Langerhans’ Cell Histiocytosis, Eosinophilic granuloma, solitary bone lesion