朗格汉斯细胞组织细胞增多症：一种神秘的疾病。
Langerhans cell histiocytosis: An enigmatic disease.
作者信息
Jain Anubha, Kumar Sushil, Aggarwal Priyanka, Kumar Mohan, Gupta Vineeta
机构信息
Department of Pediatrics, Division of Hematology-Oncology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India.
出版信息
South Asian J Cancer. 2019 Jul-Sep;8(3):183-185. doi: 10.4103/sajc.sajc_211_18.
BACKGROUND
Langerhans cell histiocytosis (LCH) is a poorly understood disease with heterogeneous clinical presentation ranging from unifocal bony involvement to disseminated disease with life-threatening complications.
MATERIALS AND METHODS
The clinical profile, laboratory findings, treatment, and long-term outcome were retrieved from maintained medical records from January 2006 to January 2016 and were retrospectively analyzed. The extent of the disease was classified as per the LCH-III trial of "The Histiocyte Society." The assessment and categorization of treatment response followed LCH III trial definitions.
RESULTS
A total of 28 children with LCH were diagnosed. The age ranged between 5 months and 9 years, with a mean of 3½ years. The M: F ratio was 3:1. Single system, unifocal and multifocal bone diseases were seen in nine (32.1%) and two (7.1%) cases, respectively. Disseminated disease without risk organ involvement was seen in six (21.1%), whereas disseminated disease with risk organ involvement was seen in 11 (39.3%) cases. The most common presentation was bony involvement (19 [67.8%]), out of which 16 (88.8%) had skull involvement. During follow-up, 17 (60.7%) were in complete remission though five (17.8%) of them relapsed, but achieved second remission. Two (7.1%) were lost to follow-up. Six (21.4%) had progressive disease of which four expired and two abandoned treatment. Two (10.7%) refused the initiation of treatment.
CONCLUSION
A better understanding of the disease, early suspicion, and diagnosis can improve the outcome of patients with LCH.
背景
朗格汉斯细胞组织细胞增多症（LCH）是一种了解较少的疾病，临床表现具有异质性，范围从单灶性骨受累到伴有危及生命并发症的播散性疾病。
材料与方法
从2006年1月至2016年1月保存的病历中检索临床资料、实验室检查结果、治疗情况及长期预后，并进行回顾性分析。疾病范围根据组织细胞协会的LCH-III试验进行分类。治疗反应的评估和分类遵循LCH III试验的定义。
结果
共诊断出28例LCH患儿。年龄在5个月至9岁之间，平均为3.5岁。男女比例为3:1。单系统、单灶性和多灶性骨疾病分别见于9例（32.1%）和2例（7.1%）。无危险器官受累的播散性疾病见于6例（21.1%），而有危险器官受累的播散性疾病见于11例（39.3%）。最常见的表现是骨受累（19例[67.8%]），其中16例（88.8%）有颅骨受累。随访期间，17例（60.7%）完全缓解，其中5例（17.8%）复发，但再次缓解。2例（7.1%）失访。6例（21.4%）病情进展，其中4例死亡，2例放弃治疗。2例（约10.7%）拒绝开始治疗。
结论
更好地了解该疾病、早期怀疑和诊断可改善LCH患者的预后。
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