Ma Jing, Jiang Yongfang, Chen Xiangyu, Gong Guozhong
Department of Hepatitis Diseases, Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, P.R. China.
Oncol Lett. 2014 May;7(5):1602-1604. doi: 10.3892/ol.2014.1928. Epub 2014 Feb 28.
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which pathological Langerhans cells accumulate in a variety of organs. LCH usually affects the bone, skin and lymph nodes of children; however, LCH occasionally affects vital organs, including the liver, spleen and pituitary gland. The present study reports a case of an adult LCH patient with marked liver damage, splenomegaly and pituitary damage treated using a new therapeutic strategy. This case was misdiagnosed as liver cancer and pituitary tumor on the basis of abdominal ultrasound, abdominal magnetic resonance imaging (MRI) and head MRI. The final diagnosis was established by identifying the proliferation of cluster of differentiation 1a-positive LCs in liver tissues. A new regimen of combined 12-week therapy of prednisolone/desmopressin/vincristine and 10 months of maintenance therapy of prednisolone/vinblastine/6-mercaptopurine improved symptoms, liver function and blood cell tests.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的增殖性疾病，其中病理性朗格汉斯细胞在多种器官中积聚。LCH通常影响儿童的骨骼、皮肤和淋巴结；然而，LCH偶尔也会影响重要器官，包括肝脏、脾脏和垂体。本研究报告了一例成年LCH患者，采用新的治疗策略治疗，出现明显的肝损伤、脾肿大和垂体损伤。根据腹部超声、腹部磁共振成像（MRI）和头部MRI，该病例被误诊为肝癌和垂体瘤。通过鉴定肝组织中分化簇1a阳性LCs的增殖确定了最终诊断。泼尼松龙/去氨加压素/长春新碱联合12周治疗和泼尼松龙/长春碱/6-巯基嘌呤10个月维持治疗的新方案改善了症状、肝功能和血细胞检查结果。
