A multicenter single-arm clinical study of Chinese children’s cancer group-acute promyelocytic leukemia-2017 (CCCG-APL-2017) protocol

Abstract The Realgar-Indigo Naturalis formula (RIF) is a proprietary Chinese medicine, which is one of the important drugs in the treatment of pediatric acute promyelocytic leukemia (APL). However, the dose of RIF in clinical application is not uniform and the long-term effectiveness and safety of c...

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Main Authors: Lixian Chang, Ju Gao, Xiaoying Lei, Yingyi He, Shuquan Zhuang, Chunhuai Li, Kaizhi Weng, Lingzhen Wang, Xia Guo, Qihui Liu, Pengfei Wang, Yong Zhuang, Mei Yan, Wei Liu, Hui Chen, Min Zhang, Shuhong Shen, Xiaofan Zhu, Xiuli Ju, Li Zhang, Zhuo Wang
Format: Article
Language:English
Published: Nature Publishing Group 2025-08-01
Series:Signal Transduction and Targeted Therapy
Online Access:https://doi.org/10.1038/s41392-025-02353-1
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author Lixian Chang
Ju Gao
Xiaoying Lei
Yingyi He
Shuquan Zhuang
Chunhuai Li
Kaizhi Weng
Lingzhen Wang
Xia Guo
Qihui Liu
Pengfei Wang
Yong Zhuang
Mei Yan
Wei Liu
Hui Chen
Min Zhang
Shuhong Shen
Xiaofan Zhu
Xiuli Ju
Li Zhang
Zhuo Wang
author_facet Lixian Chang
Ju Gao
Xiaoying Lei
Yingyi He
Shuquan Zhuang
Chunhuai Li
Kaizhi Weng
Lingzhen Wang
Xia Guo
Qihui Liu
Pengfei Wang
Yong Zhuang
Mei Yan
Wei Liu
Hui Chen
Min Zhang
Shuhong Shen
Xiaofan Zhu
Xiuli Ju
Li Zhang
Zhuo Wang
author_sort Lixian Chang
collection DOAJ
description Abstract The Realgar-Indigo Naturalis formula (RIF) is a proprietary Chinese medicine, which is one of the important drugs in the treatment of pediatric acute promyelocytic leukemia (APL). However, the dose of RIF in clinical application is not uniform and the long-term effectiveness and safety of combining RIF with all-trans retinoic acid (ATRA) in a larger population of pediatric APL patients remains undocumented. We conducted a multicenter single-arm clinical trial (ChiCTR-OIC-16010014) in China. Individuals newly diagnosed with APL were treated with CCCG-APL-2017 protocol which is based on RIF and ATRA in consolidation. The event-free survival (EFS) and overall survival (OS) outcomes were evaluated. We recruited 200 patients diagnosed with APL. The six-year OS rate was 100% in the low-risk (LR) group and 97.6% in the high-risk (HR) group. The six-year EFS rate was 98.3% in the LR group and 97.6% in the HR group. Plasma levels of arsenic remained stable after the administration of RIF at a dosage of 60 mg/kg/d for seven days and returned to baseline levels within fourteen days after discontinuation of RIF administration, which is consistent with a concentration of 135 mg/d/kg. Furthermore, controlling white blood cells (WBC) to maintain levels at or below 30 × 109/L during induction therapy can decrease the incidence of induced differentiation syndrome (DS) or alleviate its symptoms. Our study demonstrated that the CCCG-APL-2017 protocol, which combines RIF with ATRA, is both effective and safe in treating children with APL.
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spelling doaj-art-8b6dc52d8a82435bb9820fc0bca688f72025-08-24T11:54:01ZengNature Publishing GroupSignal Transduction and Targeted Therapy2059-36352025-08-011011910.1038/s41392-025-02353-1A multicenter single-arm clinical study of Chinese children’s cancer group-acute promyelocytic leukemia-2017 (CCCG-APL-2017) protocolLixian Chang0Ju Gao1Xiaoying Lei2Yingyi He3Shuquan Zhuang4Chunhuai Li5Kaizhi Weng6Lingzhen Wang7Xia Guo8Qihui Liu9Pengfei Wang10Yong Zhuang11Mei Yan12Wei Liu13Hui Chen14Min Zhang15Shuhong Shen16Xiaofan Zhu17Xiuli Ju18Li Zhang19Zhuo Wang20State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical CollegeWest China Second University Hospital, Sichuan UniversityChildren’s Hospital of Chongqing Medical UniversityDepartment of Pediatric Hematology/Oncology, Guangzhou Women and Children’s Medical Center, Guangzhou Medical UniversityQuanzhou First Hospital Affiliated to Fujian Medical UniversityDepartment of Pediatric Hematology, Children’s Medical Center, The First Hospital of Jilin UniversityDepartment of Pediatric Hematology, Rheumatology and Nephrology, Zhangzhou Municipal Hospital of Fujian ProvinceThe affiliated hospital of Qingdao UniversityWest China Second University Hospital, Sichuan UniversityChildren’s Hospital of Chongqing Medical UniversityDepartment of Pediatric Hematology/Oncology, Guangzhou Women and Children’s Medical Center, Guangzhou Medical UniversityDepartment of Pediatrics, Qilu Hospital of Shandong UniversityThe First Affiliated Hospital of Xinjiang Medical UniversityChildren’s Hospital Affiliated to Zhengzhou UniversityTianjin Children’s HospitalNorthwest women’s and children’s HospitalDepartment of Hematology/Oncology, Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University, National Health Committee Key Laboratory of Pediatric Hematology & OncologyState Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical CollegeDepartment of Pediatrics, Qilu Hospital of Shandong UniversityState Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical CollegeDepartment of Hematology/Oncology, Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University, National Health Committee Key Laboratory of Pediatric Hematology & OncologyAbstract The Realgar-Indigo Naturalis formula (RIF) is a proprietary Chinese medicine, which is one of the important drugs in the treatment of pediatric acute promyelocytic leukemia (APL). However, the dose of RIF in clinical application is not uniform and the long-term effectiveness and safety of combining RIF with all-trans retinoic acid (ATRA) in a larger population of pediatric APL patients remains undocumented. We conducted a multicenter single-arm clinical trial (ChiCTR-OIC-16010014) in China. Individuals newly diagnosed with APL were treated with CCCG-APL-2017 protocol which is based on RIF and ATRA in consolidation. The event-free survival (EFS) and overall survival (OS) outcomes were evaluated. We recruited 200 patients diagnosed with APL. The six-year OS rate was 100% in the low-risk (LR) group and 97.6% in the high-risk (HR) group. The six-year EFS rate was 98.3% in the LR group and 97.6% in the HR group. Plasma levels of arsenic remained stable after the administration of RIF at a dosage of 60 mg/kg/d for seven days and returned to baseline levels within fourteen days after discontinuation of RIF administration, which is consistent with a concentration of 135 mg/d/kg. Furthermore, controlling white blood cells (WBC) to maintain levels at or below 30 × 109/L during induction therapy can decrease the incidence of induced differentiation syndrome (DS) or alleviate its symptoms. Our study demonstrated that the CCCG-APL-2017 protocol, which combines RIF with ATRA, is both effective and safe in treating children with APL.https://doi.org/10.1038/s41392-025-02353-1
spellingShingle Lixian Chang
Ju Gao
Xiaoying Lei
Yingyi He
Shuquan Zhuang
Chunhuai Li
Kaizhi Weng
Lingzhen Wang
Xia Guo
Qihui Liu
Pengfei Wang
Yong Zhuang
Mei Yan
Wei Liu
Hui Chen
Min Zhang
Shuhong Shen
Xiaofan Zhu
Xiuli Ju
Li Zhang
Zhuo Wang
A multicenter single-arm clinical study of Chinese children’s cancer group-acute promyelocytic leukemia-2017 (CCCG-APL-2017) protocol
Signal Transduction and Targeted Therapy
title A multicenter single-arm clinical study of Chinese children’s cancer group-acute promyelocytic leukemia-2017 (CCCG-APL-2017) protocol
title_full A multicenter single-arm clinical study of Chinese children’s cancer group-acute promyelocytic leukemia-2017 (CCCG-APL-2017) protocol
title_fullStr A multicenter single-arm clinical study of Chinese children’s cancer group-acute promyelocytic leukemia-2017 (CCCG-APL-2017) protocol
title_full_unstemmed A multicenter single-arm clinical study of Chinese children’s cancer group-acute promyelocytic leukemia-2017 (CCCG-APL-2017) protocol
title_short A multicenter single-arm clinical study of Chinese children’s cancer group-acute promyelocytic leukemia-2017 (CCCG-APL-2017) protocol
title_sort multicenter single arm clinical study of chinese children s cancer group acute promyelocytic leukemia 2017 cccg apl 2017 protocol
url https://doi.org/10.1038/s41392-025-02353-1
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