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Abstract：
BACKGROUND: The pediatric pulmonary multisystem Langerhans cell histiocytosis (PPM LCH) is associated with either low risk or high risk organ(s). The nodulo-cystic lung lesions although pathognomonic, yet are very variable in severity and remain a source of controversy in certifying pulmonary LCH diagnosis. The study aimed to examine the prognostic value of clinical respiratory manifestations and radiological lung lesions severity. This is through associating a CT chest triad of bilateral, extensive and diffuse lesions. It is a retrospective study of 350 LCH patients who received systemic treatment at Children\'s Cancer Hospital Egypt during the period from 2007 to 2020.
RESULTS: Sixty-seven patients (67/350-19.1%) had PPM LCH at presentation. Severe lung lesions were present in 24 of them. The median follow-up period was 61 months (IQR: 3.4-8.3). The 5-year overall survival (OS) and event free survival (EFS) was 89% and 56.6% respectively. The EFS, for severe radiological lesions triad was 38% ± 20.7 versus 66% ± 16.2 for non-severe lesions triad p 0.002, while for presence of chest X-ray changes 27% ± 22.344 versus absence of chest X ray changes 66% ± 14.7 p 0.001, for clinical respiratory manifestations 13% ± 13.9 versus none 62% ± 22.9 p < 0.001, for RO- with severe lung lesions 47% ± 30.4 versus RO- without severe lung lesions 69% ± 5.9 p 0.04. There was a tendency for the independent prognostic impact of severe lung involvement; aHR = 1.7 (95% CI 0.92-3.13, p = 0.09).
CONCLUSIONS: Although the lung is a low -risk organ per se in LCH, our study demonstrates a non negligeable prognostic impact of severe lung involvement in the risk stratification of pediatric LCH. This warrants further study and external validation.
RESULTS: Sixty-seven patients (67/350-19.1%) had PPM LCH at presentation. Severe lung lesions were present in 24 of them. The median follow-up period was 61 months (IQR: 3.4-8.3). The 5-year overall survival (OS) and event free survival (EFS) was 89% and 56.6% respectively. The EFS, for severe radiological lesions triad was 38% ± 20.7 versus 66% ± 16.2 for non-severe lesions triad p 0.002, while for presence of chest X-ray changes 27% ± 22.344 versus absence of chest X ray changes 66% ± 14.7 p 0.001, for clinical respiratory manifestations 13% ± 13.9 versus none 62% ± 22.9 p < 0.001, for RO- with severe lung lesions 47% ± 30.4 versus RO- without severe lung lesions 69% ± 5.9 p 0.04. There was a tendency for the independent prognostic impact of severe lung involvement; aHR = 1.7 (95% CI 0.92-3.13, p = 0.09).
CONCLUSIONS: Although the lung is a low -risk organ per se in LCH, our study demonstrates a non negligeable prognostic impact of severe lung involvement in the risk stratification of pediatric LCH. This warrants further study and external validation.
摘要：
背景：小儿肺多系统朗格汉斯细胞组织细胞增生症（PPMLCH）与低风险或高风险器官有关。结节囊性肺病变虽然是病理性的，然而，严重程度差异很大，在证明肺LCH诊断方面仍然存在争议。该研究旨在检查临床呼吸道表现和放射性肺部病变严重程度的预后价值。这是通过关联双侧CT胸部三联征，广泛和弥漫性病变。这是一项对2007年至2020年期间在埃及儿童肿瘤医院接受全身治疗的350名LCH患者的回顾性研究。
结果：67例患者（67/350-19.1%）出现PPMLCH。其中24例存在严重的肺部病变。中位随访期为61个月(IQR:3.4-8.3)。5年总生存率（OS）和无事件生存率（EFS）分别为89％和56.6％。EFS,对于严重的放射学病变三联征，非严重病变三联征分别为38％±20.7和66％±16.2p0.002，而对于胸部X线变化的存在，为27％±22.344与不存在胸部X线变化的66％±14.7p0.001，对于临床呼吸道表现，为13％±13.9与无62％±22.9p<0.001，对于有严重肺部病变的RO-有47％±30.4严重肺部受累对预后有独立影响的趋势；aHR=1.7（95％CI0.92-3.13，p=0.09）。
结论：尽管肺本身是LCH的低风险器官，我们的研究表明,在小儿LCH的危险分层中,严重肺部受累对预后的影响不可忽视.这需要进一步研究和外部验证。
结果：67例患者（67/350-19.1%）出现PPMLCH。其中24例存在严重的肺部病变。中位随访期为61个月(IQR:3.4-8.3)。5年总生存率（OS）和无事件生存率（EFS）分别为89％和56.6％。EFS,对于严重的放射学病变三联征，非严重病变三联征分别为38％±20.7和66％±16.2p0.002，而对于胸部X线变化的存在，为27％±22.344与不存在胸部X线变化的66％±14.7p0.001，对于临床呼吸道表现，为13％±13.9与无62％±22.9p<0.001，对于有严重肺部病变的RO-有47％±30.4严重肺部受累对预后有独立影响的趋势；aHR=1.7（95％CI0.92-3.13，p=0.09）。
结论：尽管肺本身是LCH的低风险器官，我们的研究表明,在小儿LCH的危险分层中,严重肺部受累对预后的影响不可忽视.这需要进一步研究和外部验证。
