Onal Cem, Oymak Ezgi, Reyhan Mehmet, Canpolat Tuba, Ozyilkan Ozgur
Department of Radiation Oncology, Baskent University Faculty of Medicine, Adana, Turkey.
Adana Research and Treatment Centre, Department of Radiation Oncology, Baskent University Faculty of Medicine, 01120, Adana, Turkey.
Jpn J Radiol. 2015 Sep;33(9):603-6. doi: 10.1007/s11604-015-0466-6. Epub 2015 Jul 26.
Langerhans' cell histiocytosis (LCH) is a proliferative disorder of Langerhans cells, which is seen extremely rarely in adults. Conventional imaging modalities, such as skeletal surveys and bone scans, were accepted to be standard methods for diagnosis; however, 18-fluorodeoxyglucose positron emission tomography (PET-CT) has been increasingly used.
We report on a 33-year-old female patient with disseminated LCH treated with radiotherapy and systemic chemotherapy where PET-CT has been used for defining the extent of the disease, RT planning and assessment of treatment response during follow-up.
The patient was treated with 24 Gy 3-dimensional conformal radiotherapy (RT), given as 2 Gy a day, 5 days a week. The patient was also treated with systemic prednisolone 20 mg/m(2), concurrently. The chemotherapeutic regimen switched to cytosine-arabinoside with a dose of 100 mg/m(2) subcutaneously daily for 4 days, vincristine 1.5 mg/m(2) given on the 1st day and prednisolone 20 mg/m(2) for 4 cycles. After local RT with adjuvant chemotherapy, the patient was alive for 54 months and remained disease-free at last visit.
RT is a treatment choice in multi-system LCH as well as solitary lesions. Low-dose RT is adequate to control large masses of LCH including soft tissue and lymph nodes.
朗格汉斯细胞组织细胞增多症（LCH）是一种朗格汉斯细胞的增殖性疾病，在成人中极为罕见。传统的成像方式，如骨骼检查和骨扫描，曾被认为是诊断的标准方法；然而，18-氟脱氧葡萄糖正电子发射断层扫描（PET-CT）的使用越来越多。
我们报告了一名33岁的女性播散性LCH患者，接受了放疗和全身化疗，其中PET-CT用于确定疾病范围、放疗计划以及随访期间治疗反应的评估。
患者接受了24 Gy的三维适形放疗（RT），每天2 Gy，每周5天。患者同时还接受了20 mg/m²的全身泼尼松龙治疗。化疗方案改为阿糖胞苷，剂量为100 mg/m²皮下注射，每日1次，共4天，第1天给予长春新碱1.5 mg/m²，泼尼松龙20 mg/m²，共4个周期。在局部放疗联合辅助化疗后，患者存活了54个月，最后一次随访时仍无疾病。
放疗是多系统LCH以及孤立性病变的一种治疗选择。低剂量放疗足以控制包括软组织和淋巴结在内的大块LCH。
