Langerhans Cell Histiocytosis: A Population-based Study of Anatomical Distribution and Treatment Patterns,Journal of Bone Oncology

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Langerhans Cell Histiocytosis: A Population-based Study of Anatomical Distribution and Treatment Patterns
Journal of Bone Oncology ( IF 3.1 ) Pub Date : 2022-09-27 , DOI: 10.1016/j.jbo.2022.100454
Xianglin Hu ₁ , Ilia N Buhtoiarov ₂ , Chunmeng Wang ₁ , Zhengwang Sun ₁ , Qinyuan Zhu ₃ , Wending Huang ₁ , Wangjun Yan ₁ , Yangbai Sun ₁

Affiliation

Background

Langerhans cell histiocytosis (LCH) is a rare monoclonal histiocytic neoplasm. Little is known about clinical factors associated with LCH single- vs multi-system involvement at the time of diagnosis.

Methods

Data on 1549 LCH patients diagnosed between years 2010 and 2018 were extracted from the Surveillance, Epidemiology and End Results Program. Patterns of single- vs multisystem involvement were examined using multivariable logistic regression analysis. Odd ratio (OR) and 95% confidence interval (CI) were reported.

Results

968 children and adolescents (0–19 years; median: 4 years) and 581 adults (≥20 years; median: 49 years) were included in the analysis. Multi-system LCH was reported for 30.9 % patients. Bone marrow (BM) (OR = 3.776; 95 %CI = 1.939–7.351; P < 0.001) and lymph node (LN) (OR = 3.274; 95 %CI = 1.443–7.427; P = 0.005) involvement were most commonly associated with multi-system LCH at the time of diagnosis; similar pattern was also observed in adult patients (OR = 17.780; 95 %CI = 6.469–48.867; P < 0.001 for BM LCH; and OR = 5.156; 95 %CI = 2.131–12.471; P < 0.001 for LN LCH). Among pediatric patients, craniofacial osseous LCH was more likely to be treated with surgery (OR = 2.822; 95 %CI = 1.199–6.639; P = 0.018) compared to skeletal lesions in other sites, whereas vertebral body LCH was less likely to be treated with surgery (OR = 0.175; 95 %CI = 0.058–0.527; P = 0.002). In pediatric patients with bone LCH, the non-white patients were less likely to be treated surgically compared to the white patients (OR = 0.470; 95 %CI = 0.272–0.812; P = 0.007).

Conclusions

BM and LN LCH are associated with the highest risks of multi-system disease, which may require active surveillance. Furthermore, active attempts are needed to mitigate the racial disparity in surgery utilization in pediatric patients with skeletal LCH.

中文翻译：

朗格汉斯细胞组织细胞增生症：基于人群的解剖分布和治疗模式研究

背景

朗格汉斯细胞组织细胞增生症（LCH）是一种罕见的单克隆组织细胞肿瘤。在诊断时，对与 LCH 单系统和多系统受累相关的临床因素知之甚少。

方法

从监测、流行病学和最终结果计划中提取了 2010 年至 2018 年间诊断的 1549 名 LCH 患者的数据。使用多变量逻辑回归分析检查单系统与多系统受累的模式。报告了优势比 (OR) 和 95% 置信区间 (CI)。

结果

968 名儿童和青少年（0-19 岁；中位数：4 岁）和 581 名成人（≥20 岁；中位数：49 岁）被纳入分析。30.9% 的患者报告了多系统 LCH。骨髓 (BM) (OR = 3.776；95 %CI = 1.939–7.351；P < 0.001) 和淋巴结 (LN) (OR = 3.274；95 %CI = 1.443–7.427；P = 0.005) 受累最常见诊断时使用多系统 LCH；在成年患者中也观察到类似的模式（OR = 17.780；95 %CI = 6.469–48.867；BM LCH 的 P < 0.001；OR = 5.156；95 %CI = 2.131–12.471；LN LCH 的 P < 0.001）。在儿科患者中，与其他部位的骨骼病变相比，颅面骨性 LCH 更可能接受手术治疗（OR = 2.822；95 %CI = 1.199–6.639；P = 0.018），而椎体 LCH 不太可能接受手术治疗手术（OR = 0.175；95%CI = 0.058–0.527；P = 0.002）。在患有骨性 LCH 的儿科患者中，与白人患者相比，非白人患者接受手术治疗的可能性较小（OR = 0.470；95 %CI = 0.272–0.812；P = 0.007）。