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ORIGINAL RESEARCH article

Front. Med.
Sec. Hematology
Volume 11 - 2024 | doi: 10.3389/fmed.2024.1452003

Clinical features and prognostic factors of pediatric Langerhans cell histiocytosis: a single-center retrospective study

Provisionally accepted
Yunfeng Lu Yunfeng Lu Liying Liu Liying Liu Qi Wang Qi Wang Bingju Liu Bingju Liu Ping Zhao Ping Zhao Guotao Guan Guotao Guan Yunpeng Dai Yunpeng Dai *
  • Department of Pediatric hematology and oncology,Shandong Provincial Hospital affiliated to Shandong First Medical University, Jinan, China

The final, formatted version of the article will be published soon.

    Purpose: To retrospectively evaluate the clinical features and prognostic factors of pediatric LCH patients treated in a single center of China.Methods: Pediatric LCH cases were treated following the SD-LCH protocol at the Affiliated Provincial Hospital of Shandong First Medical University in Jinan, China. An analysis was conducted on 82 recently identified LCH cases to retrospectively evaluate the initial symptoms, therapeutic alternatives, and extended results. Follow-ups were conducted until July 31, 2023.The median age at diagnosis was 2 (0.25 -12) years. 42 (51.2%) were SS-LCH, and 40 (48.8%) were MS-LCH. The most common organ involved was bone (82.9%). Over the 16-year follow-up period, the 5-year EFS and OS rates were 75.2±5% and 90.9±3.3%, respectively. The cumulative reactivation rate was 23.2%. The 5-year EFS rate in SS-LCH and MS-LCH patients were 90.2±4.6% and 58.8±8.3%, and the 5-year OS rate in SS-LCH and MS-LCH patients were 90.2±4.6% and 81.2±6.5%, respectively. The 5-year OS and EFS rate in RO+ LCH and RO-LCH patients were 79.5±7.5%, 53.8±9.6% and 87.5±11.7%, 76.2±14.8%, insignificantly. Multivariate Cox regression showed that liver involvement predicted poor EFS and hematological system involvement was an independent prognostic factor for OS. Detection of the BRAF V600E mutation and targeted therapy significantly improved the prognosis post-2017.Conclusion: Liver or hematological system involvement indicates a poor prognosis, and the SD-LCH protocol improves prognosis for pediatric LCH patients.

    Keywords: langerhans cell histiocytosis, chemotherapy, pediatric oncology, BRAF V600E inhibitor, prognosis

    Received: 24 Jun 2024; Accepted: 18 Dec 2024.

    Copyright: © 2024 Lu, Liu, Wang, Liu, Zhao, Guan and Dai. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Yunpeng Dai, Department of Pediatric hematology and oncology,Shandong Provincial Hospital affiliated to Shandong First Medical University, Jinan, China

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.