Chang Long, Wang Luo, Liu Zheng-Zheng, Lang Min, Lin He, Liu Xiao-Yan, Duan Ming-Hui, Zhou Dao-Bin, Cao Xin-Xin
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, People's Republic of China.
State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Beijing, People's Republic of China.
Ann Hematol. 2025 Jan;104(1):65-74. doi: 10.1007/s00277-024-06167-z. Epub 2025 Jan 7.
Langerhans cell histiocytosis (LCH) is a heterogeneous histiocytosis with various pulmonary manifestations and imaging. We aim to evaluate the pulmonary response of LCH by high resolution chest computed tomography (HRCT) through continuous follow-up.We conducted a retrospective analysis of 73 adult LCH patients with pulmonary involvement. HRCT response was assessed by the change of HRCT global score (nodule score plus cyst score) between the baseline and after therapy. Among them, 69 patients (94.5%) had multi-system LCH with pulmonary involvement. 42 patients received methotrexate and cytarabine regimen, 15 received cytarabine monotherapy, 7 underwent target therapy. 14 (19.2%) achieved complete response (CR) and 45 (61.6%) achieved partial response (PR). The mean global lung-lesion score decreased from 12.2 to 10.6. The mean nodule score decreased from 4.7 to 4.1 and the mean cyst score decreased from 7.4 to 6.5. Overall, 25 (34.2%) exhibited an HRCT response and 3 (4.1%) had HRCT progression, while 45 (61.6%) maintained the same. Among patients with CR or PR, 57.1% and 40.5%, respectively, experienced an HRCT response, whereas no patient with SD or PD had an HRCT response. Multivariable analyses revealed that patients who received low dose cytarabine regimen and those with HRCT score ≥ 10 predicted a shorter PFS. Long Chang, Luo Wang, and Zheng-zheng Liu contributed equally to this work.
朗格汉斯细胞组织细胞增多症（LCH）是一种具有多种肺部表现和影像学特征的异质性组织细胞增多症。我们旨在通过高分辨率胸部计算机断层扫描（HRCT）连续随访来评估LCH的肺部反应。我们对73例有肺部受累的成年LCH患者进行了回顾性分析。通过基线和治疗后HRCT整体评分（结节评分加囊肿评分）的变化来评估HRCT反应。其中，69例（94.5%）患者有多系统LCH伴肺部受累。42例患者接受甲氨蝶呤和阿糖胞苷方案，15例接受阿糖胞苷单药治疗，7例接受靶向治疗。14例（19.2%）达到完全缓解（CR），45例（61.6%）达到部分缓解（PR）。肺部病变平均整体评分从12.2降至10.6。平均结节评分从4.7降至4.1，平均囊肿评分从7.4降至6.5。总体而言，25例（34.2%）出现HRCT反应，3例（4.1%）有HRCT进展，45例（61.6%）维持不变。在CR或PR患者中，分别有57.1%和40.5%出现HRCT反应，而疾病稳定（SD）或疾病进展（PD）的患者无HRCT反应。多变量分析显示，接受低剂量阿糖胞苷方案的患者和HRCT评分≥10的患者预测无进展生存期（PFS）较短。龙畅、王罗、刘正正对此工作贡献相同。
