Fabozzi Antonio, Paciucci Gianluca, de Rose Giulia, Romiti Roberto, Palumbo Giovanna, Paone Gregorino, Bonini Matteo, Palange Paolo
Pulmonology Unit, Department of Public Health and Infectious Diseases, Policlinico Umberto I, "Sapienza" University of Rome, 00185 Rome, Italy.
Division of Hematology, Department of Translational and Precision Medicine, Policlinico Umberto I, "Sapienza" University of Rome, 00185 Rome, Italy.
Life (Basel). 2025 Aug 7;15(8):1258. doi: 10.3390/life15081258.
Langerhans Cell Histiocytosis (LCH) is a rare histiocytic hematological disorder that frequently involves the lungs. Due to a lack of data about sex-related differences in LCH, the aim of this study is to evaluate sex-related differences in pulmonary function in a cohort of patients with LCH.
We retrospectively analyzed data from 79 adult patients diagnosed with LCH. Demographic, clinical, and spirometric data were collected and compared by sex. Continuous variables were analyzed using the Mann-Whitney test and categorical variables were analyzed with the Chi-square test.
Out of 79 patients, 47 (59.5%) were females and 32 (40.5%) were males. Women showed significantly lower diffusing capacity of the lungs for carbon monoxide (DLCO%) and lower diffusing capacity of the lungs for carbon monoxide per unit of alveolar volume (DLCO/VA%) compared to men. Females showed a trend toward lower small airway indices, including maximal expiratory flow at 25 (MEF%) and forced expiratory flow at 25-75% (FEF%), though this was not statistically significant, while the residual volume-to-total lung capacity (RV/TLC) ratio was significantly higher in women. Among the functional parameters, DLCO% showed the highest accuracy (AUC 0.70) in the identification of lung involvement after multivariate regression analysis.
Our findings suggest that the combination of lower gas exchange efficiency and increased peripheral air trapping secondary to small airway involvement in female patients may reflect the presence of a distinct functional LCH phenotype in women characterized by early small airway involvement and altered ventilation-perfusion dynamics, which may influence the clinical management of these patients. Furthermore, the moderate predictive value of DLCO% for lung involvement at baseline in LCH women suggests that DLCO may contribute to the detection of LCH women with lung involvement, although it should not be considered a definitive diagnostic test without a prospective and independent external validation.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的组织细胞性血液系统疾病，常累及肺部。由于缺乏关于LCH性别差异的数据，本研究旨在评估一组LCH患者的肺功能性别差异。
我们回顾性分析了79例诊断为LCH的成年患者的数据。收集人口统计学、临床和肺功能数据，并按性别进行比较。连续变量采用曼-惠特尼检验分析，分类变量采用卡方检验分析。
79例患者中，47例（59.5%）为女性，32例（40.5%）为男性。与男性相比，女性的一氧化碳肺弥散量（DLCO%）和单位肺泡容积一氧化碳肺弥散量（DLCO/VA%）显著降低。女性的小气道指标有降低趋势，包括25%肺活量时的最大呼气流量（MEF%）和25%-75%肺活量时的用力呼气流量（FEF%），但差异无统计学意义，而女性的残气量与肺总量（RV/TLC）比值显著更高。在功能参数中，多因素回归分析后，DLCO%在识别肺部受累方面显示出最高的准确性（AUC 0.70）。
我们的研究结果表明，女性患者气体交换效率降低和继发于小气道受累的外周气体潴留增加的组合，可能反映了女性存在一种独特的功能性LCH表型，其特征为早期小气道受累和通气-灌注动力学改变，这可能影响这些患者的临床管理。此外，DLCO%对LCH女性患者基线时肺部受累的中度预测价值表明，DLCO可能有助于检测出有肺部受累的LCH女性患者，尽管在没有前瞻性和独立外部验证的情况下，不应将其视为确定性诊断试验。
