儿科肺部多系统朗格汉斯细胞囊细胞形成:肺病变的严重程度是否会影响结果?
Mohamed Sedky1,2, Seham Gohar3, Sonia Ahmed3,4
1Department of Pediatric Oncology, Children Cancer Hospital Egypt 57357 1 (11617), Sekket Al-Imam St., Al Sayyeda Zeinab, Cairo, 11617, Egypt. mohamed.sedki@57357.org.
Orphanet journal of rare diseases
|November 18, 2023
概括
在儿科肺部多系统朗格汉斯细胞囊炎 (PPM LCH) 中严重的肺部干扰显著影响患者的结果. 这项研究强调了PPM LCH风险分层中肺病变严重程度的预后价值.
科学领域:
- 儿科瘤学 儿科瘤学
- 肺部病理学 肺部病理学
- 组织细胞形成研究 组织细胞形成研究
背景情况:
- 儿科肺部多系统朗格汉斯细胞囊细胞瘤 (PPM LCH) 涉及基于器官参与的风险分层.
- 在PPM LCH中,结节囊性肺病变的严重程度各不相同,使诊断和预后评估复杂化.
- 临床呼吸道症状和放射性肺损伤严重程度的预后值需要进一步调查.
研究的目的:
- 评估PPM LCH.临床呼吸道症状的预后意义.
- 评估放射性肺病变严重程度,特别是双边,广泛和扩散病变的CT胸部三元体对患者结局的影响.
- 通过纳入肺部参与来完善儿科LCH的风险分层.
主要方法:
- 从2007年到2020年,对350名儿科LCH患者进行了系统治疗的回顾性分析.
- 临床呼吸系统症状和CT胸部成像发现 (损伤严重程度,分布) 与生存结果的相关性.
- 统计分析包括无事件生存率 (EFS) 和总生存率 (OS) 与肺部感染严重程度相关.
主要成果:
- 19.1%的患者出现了PPM LCH;24人患有严重的肺病变.
- 在患有严重放射性病变的患者中,无事件生存 (EFS) 显着较低 (38%),而非严重病变的患者则较低 (66%) (p=0.002).
- 临床呼吸道表现和胸部X射线变化也与较差的EFS相关 (分别p<0.001和p=0.001).
结论:
- 在PPM LCH中,严重的肺部参与具有显著的预后影响,尽管肺被认为是低风险器官.
- 这些发现表明,严重的肺部表现应纳入儿科LCH的风险分层.
- 建议进行进一步的研究和外部验证,以确认PPM LCH中严重肺部参与的预后价值.
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