Yağci Begül, Varan Ali, Uner Aysegül, Akyüz Canan, Büyükpamukçu Münevver
Department of Pediatric Oncology, Hacettepe University Institute of Oncology, Ankara, Turkey.
Pediatr Blood Cancer. 2008 Dec;51(6):833-5. doi: 10.1002/pbc.21690.
Langerhans cell histiocytosis (LCH) is a rare disorder characterized by clonal expansion of antigen presenting Langerhans cells. Different clinical features can be seen according to the involved organs and systems. Multisystem disease with organ dysfunction is more common in infants, whereas single system disease is usually observed in older children. The disease can affect any system or organ throughout the body. Thymus is a rarely involvement site reported in LCH and usually is accompanied by skin, bone or lung disease. Here we report a 12-year-old male with thymic involvement by LCH clinically mimicking lymphoma.
朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的疾病,其特征为抗原呈递朗格汉斯细胞的克隆性增殖。根据受累的器官和系统不同,可出现不同的临床特征。伴有器官功能障碍的多系统疾病在婴儿中更为常见,而单系统疾病通常见于大龄儿童。该疾病可累及全身任何系统或器官。胸腺是LCH中报道较少的受累部位,通常伴有皮肤、骨骼或肺部疾病。在此,我们报告一例12岁男性,其胸腺受LCH累及,临床上酷似淋巴瘤。