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患有朗格汉斯细胞细胞症的儿童的肺部参与

Çağrı Coşkun¹, Tezer Kutluk¹, Bilgehan Yalçın¹

¹Department of Pediatric Oncology, Faculty of Medicine, Hacettepe University, Ankara, Türkiye.

The Turkish journal of pediatrics

|July 18, 2024

概括

儿童肺部朗格汉斯细胞囊细胞瘤 (pLCH) 通常涉及多个器官,需要谨慎管理. 治疗结果显示中等的长期存活率,强调需要继续研究.

科学领域:

儿科肺病学儿科肺病学
血液学-瘤学血液学-瘤学
罕见疾病罕见疾病

背景情况:

肺部朗格汉斯细胞囊细胞症 (pLCH) 是一种罕见的儿科肺部疾病.
pLCH经常与多系统的朗格汉斯细胞囊细胞症 (LCH) 相关.

研究的目的:

为了研究儿科pLCH的临床特征.
评估诊断为pLCH的儿童的治疗结果.

主要方法:

临床,放射和治疗数据的回顾性审查.
分析了37名在1974年至2022年间被诊断患有pLCH的儿科患者.

主要成果:

pLCH占LCH病例的10%,中位数年龄为1.8岁.
29.7%的患者有呼吸道症状;成像显示出多种肺部异常.
十年无事件生存率 (EFS) 为47.8%,总生存率 (OS) 为63.3%.

结论:

pLCH是LCH的一个显著表现,通常涉及多个器官系统.
有效的管理策略对于减轻儿科pLCH的发病率和死亡率至关重要.

关键词:

兰格拉汉斯细胞基细胞分裂孩子们的孩子们的孩子们的孩子们.肺部卷入肺部卷入

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