朗格汉斯细胞组织细胞增多症：单中心经验
Langerhan's cell histiocytosis: A single institutional experience.
作者信息
Singh Tejinder, Satheesh C T, Appaji L, Aruna Kumari B S, Mamatha H S, Giri G V, Rama Rao Clementina
机构信息
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore - 560 030, India.
出版信息
Indian J Med Paediatr Oncol. 2010 Apr;31(2):51-3. doi: 10.4103/0971-5851.71655.
BACKGROUND
Langerhans cell histiocytosis (LCH) is a disease that primarily affects bone but can be associated with a clinical spectrum that ranges from a solitary bone lesion with a favorable natural history to a multisystem, life-threatening disease process.
AIM
We analyzed our single institutional experience of managing children with LCH.
SETTINGS AND DESIGN
A total of 40 children of LCH, managed in tertiary cancer center in South India in the period from 2001 to 2005, were evaluated retrospectively.
MATERIALS AND METHODS
Clinicopathological features, laboratory findings, treatment modalities and long-term outcome were analyzed.
RESULTS
Children were aged between 2 months and 12 years, with a mean of 3 years. Majority of the children were below 5 years of age. Group B constituted a bulk of children. Disseminated cases were less (five patients). Liver function dysfunction was seen in four (10%) children. Pulmonary interstitial infiltrates were seen in two (5%) cases. Diabetes insipidus manifested in three patients. There was one death.
CONCLUSION
A better understanding of the etiology and pathogenesis of LCH will result in more directed and efficacious treatment regimens.
背景
朗格汉斯细胞组织细胞增多症（LCH）是一种主要累及骨骼的疾病，但可伴有一系列临床症状，从具有良好自然病程的孤立性骨病变到多系统、危及生命的疾病过程。
目的
我们分析了我们在管理LCH患儿方面的单机构经验。
设置与设计
对2001年至2005年期间在印度南部三级癌症中心接受治疗的40例LCH患儿进行回顾性评估。
材料与方法
分析临床病理特征、实验室检查结果、治疗方式和长期预后。
结果
患儿年龄在2个月至12岁之间，平均年龄为3岁。大多数患儿年龄在5岁以下。B组患儿占大多数。播散性病例较少（5例）。4例（10%）患儿出现肝功能障碍。2例（5%）病例出现肺间质浸润。3例患儿出现尿崩症。有1例死亡。
结论
对LCH的病因和发病机制有更好的了解将导致更有针对性和有效的治疗方案。
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