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. 2022 Sep 27:36:100454.
doi: 10.1016/j.jbo.2022.100454. eCollection 2022 Oct.

Langerhans Cell Histiocytosis: A Population-based Study of Anatomical Distribution and Treatment Patterns

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
Affiliations

Langerhans Cell Histiocytosis: A Population-based Study of Anatomical Distribution and Treatment Patterns

Xianglin Hu et al. J Bone Oncol. .

Abstract

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.

Keywords: Chemotherapy; Epidemiology and end results; Langerhans cell histiocytosis; Racial disparity; Surgery; Surveillance; Treatment.

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Conflict of interest statement

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Figures

None
Graphical abstract
Fig. 1
Fig. 1
Flowchart showing patient’s selection.
Fig. 2
Fig. 2
Forest plots showing factors associated with multi-system disease in pediatric (A) and adult (B) patients with LCH.
Fig. 3
Fig. 3
Forest plots showing factors associated with treatments in pediatric patients with LCH (0–19 years).
Fig. 4
Fig. 4
Forest plots showing factors associated with treatments in pediatric patients with skeletal LCH (0–19 years).
See this image and copyright information in PMC

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