Intermediate-dose immune tolerance induction outperforms with faster success, less bleeding, and no added cost in comparison with low dose: a multicenter randomized clinical trial

Background: Low-dose (LD) or intermediate-dose (MD) immune tolerance induction (ITI) is effective in children with severe hemophilia A (SHA) with high-titer inhibitors (HTIs) and is attractive in countries with economic constraints. However, high-quality evidence of their use is lacking. Objectives:...

Full description

Saved in:
Bibliographic Details
Main Authors: Zhengping Li, Zekun Li, Xiaoling Cheng, Heng Zhang, Can Yang, Qian Xu, Zhenping Chen, Yingzi Zhen, Gang Li, Guoqing Liu, Wanru Yao, Min Zhou, Jiao Jin, Jie Huang, Yongjun Fang, Liangzhi Xie, Man-Chiu Poon, Runhui Wu
Format: Article
Language:English
Published: Elsevier 2025-01-01
Series:Research and Practice in Thrombosis and Haemostasis
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2475037924003340
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1850119955384631296
author Zhengping Li
Zekun Li
Xiaoling Cheng
Heng Zhang
Can Yang
Qian Xu
Zhenping Chen
Yingzi Zhen
Gang Li
Guoqing Liu
Wanru Yao
Min Zhou
Jiao Jin
Jie Huang
Yongjun Fang
Liangzhi Xie
Man-Chiu Poon
Runhui Wu
author_facet Zhengping Li
Zekun Li
Xiaoling Cheng
Heng Zhang
Can Yang
Qian Xu
Zhenping Chen
Yingzi Zhen
Gang Li
Guoqing Liu
Wanru Yao
Min Zhou
Jiao Jin
Jie Huang
Yongjun Fang
Liangzhi Xie
Man-Chiu Poon
Runhui Wu
author_sort Zhengping Li
collection DOAJ
description Background: Low-dose (LD) or intermediate-dose (MD) immune tolerance induction (ITI) is effective in children with severe hemophilia A (SHA) with high-titer inhibitors (HTIs) and is attractive in countries with economic constraints. However, high-quality evidence of their use is lacking. Objectives: This was a multicenter randomized clinical trial comparing the efficacy, safety, and medication cost between LD-ITI and MD-ITI for SHA-HTI children. Methods: Children with SHA aged <8 years with historical/pre-ITI inhibitor titer 5 to 200 Bethesda Units/mL in 3 centers were randomized 1:1 to receive LD-ITI (recombinant factor VIII [rFVIII] 50 IU/kg every other day) or MD-ITI (rFVIII 100 IU/kg/d) from January 2022 to June 2024 (ChiCTR2200056603, https://www.chictr.org.cn). Results: Thirty-one patients (16 in MD-ITI and 15 in LD-ITI) were enrolled and followed for >24 months (median, 26.9; range, 24.0-29.5 months). The 2 groups had similar baseline clinical characteristics and similar success rates (93.8% [MD-ITI] vs 86.7% [LD-ITI]). Compared with LD-ITI, MD-ITI patients took a shorter median time to success (4.2 months vs 10.1 months) and partial success (2.7 months vs 6.6 months) and had lower mean rates for all bleeding (0.38/mo vs 1.40/mo) and joint bleeding (0.11/mo vs 0.83/mo). Between the 2 groups, although the MD-ITI group had higher rFVIII consumption (12,775 vs 7680 IU/kg), their total medication costs to success were similar (3626.49 vs 3240.38 US$/kg). Conclusion: For SHA-HTI children, the success rate and cost for MD-ITI and LD-ITI regimens were similar. MD-ITI regimen would be a priority for regions with economic constraints, considering the shorter time to success, better bleeding control, and no increase in medication cost.
format Article
id doaj-art-eaed6865edbb4c3eab4cc65bc8ba7346
institution OA Journals
issn 2475-0379
language English
publishDate 2025-01-01
publisher Elsevier
record_format Article
series Research and Practice in Thrombosis and Haemostasis
spelling doaj-art-eaed6865edbb4c3eab4cc65bc8ba73462025-08-20T02:35:30ZengElsevierResearch and Practice in Thrombosis and Haemostasis2475-03792025-01-019110263910.1016/j.rpth.2024.102639Intermediate-dose immune tolerance induction outperforms with faster success, less bleeding, and no added cost in comparison with low dose: a multicenter randomized clinical trialZhengping Li0Zekun Li1Xiaoling Cheng2Heng Zhang3Can Yang4Qian Xu5Zhenping Chen6Yingzi Zhen7Gang Li8Guoqing Liu9Wanru Yao10Min Zhou11Jiao Jin12Jie Huang13Yongjun Fang14Liangzhi Xie15Man-Chiu Poon16Runhui Wu17Hemophilia Comprehensive Care Center, Hematology Center, Beijing Key Laboratory of Pediatric Hematology-Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, China; Hematologic Disease Laboratory, Hematology Center, Beijing Key Laboratory of Pediatric Hematology-Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Pediatric Research Institute, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, ChinaHemophilia Comprehensive Care Center, Hematology Center, Beijing Key Laboratory of Pediatric Hematology-Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, China; Hematologic Disease Laboratory, Hematology Center, Beijing Key Laboratory of Pediatric Hematology-Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Pediatric Research Institute, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, ChinaDepartment of Pharmacy, Beijing Children’s Hospital, National Center for Children’s Health, Capital Medical University, Beijing, ChinaDepartment of Hematology and Oncology, Children's Hospital of Nanjing Medical University, Nanjing, ChinaDepartment of Hematology and Oncology, The Affiliated Hospital of Guizhou Medical University, Guizhou, ChinaDepartment of Hematology &amp; Oncology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, ChinaHematologic Disease Laboratory, Hematology Center, Beijing Key Laboratory of Pediatric Hematology-Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Pediatric Research Institute, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, ChinaHemophilia Comprehensive Care Center, Hematology Center, Beijing Key Laboratory of Pediatric Hematology-Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, ChinaHematologic Disease Laboratory, Hematology Center, Beijing Key Laboratory of Pediatric Hematology-Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Pediatric Research Institute, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, ChinaHemophilia Comprehensive Care Center, Hematology Center, Beijing Key Laboratory of Pediatric Hematology-Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, ChinaHemophilia Comprehensive Care Center, Hematology Center, Beijing Key Laboratory of Pediatric Hematology-Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, ChinaDepartment of Hematology &amp; Oncology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, ChinaDepartment of Hematology and Oncology, The Affiliated Hospital of Guizhou Medical University, Guizhou, ChinaDepartment of Hematology and Oncology, Children's Hospital of Nanjing Medical University, Nanjing, ChinaDepartment of Hematology and Oncology, Children's Hospital of Nanjing Medical University, Nanjing, ChinaBeijing Engineering Research Center of Protein and Antibody, SinoCellTech Ltd, Beijing, China; Liangzhi Xie, Beijing Engineering Research Center of Protein and Antibody, SinoCellTech Ltd, Room 301, Building 5, No. 31 Kechuang 7th Street, Beijing Economic and Technological Development Zone, Beijing, China.Departments of Medicine, Pediatrics and Oncology, University of Calgary Cumming School of Medicine, Calgary, Alberta, Canada; Man-Chiu Poon, Cumming School of Medicine, University of Calgary, 3330 Hospital Drive NW, Calgary, AB T2N 4N1, Canada.Hemophilia Comprehensive Care Center, Hematology Center, Beijing Key Laboratory of Pediatric Hematology-Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, China; Correspondence Runhui Wu, Hemophilia Comprehensive Care Center, Hematology Center, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, 56 Nanlishi Road, West District, Beijing, China.Background: Low-dose (LD) or intermediate-dose (MD) immune tolerance induction (ITI) is effective in children with severe hemophilia A (SHA) with high-titer inhibitors (HTIs) and is attractive in countries with economic constraints. However, high-quality evidence of their use is lacking. Objectives: This was a multicenter randomized clinical trial comparing the efficacy, safety, and medication cost between LD-ITI and MD-ITI for SHA-HTI children. Methods: Children with SHA aged <8 years with historical/pre-ITI inhibitor titer 5 to 200 Bethesda Units/mL in 3 centers were randomized 1:1 to receive LD-ITI (recombinant factor VIII [rFVIII] 50 IU/kg every other day) or MD-ITI (rFVIII 100 IU/kg/d) from January 2022 to June 2024 (ChiCTR2200056603, https://www.chictr.org.cn). Results: Thirty-one patients (16 in MD-ITI and 15 in LD-ITI) were enrolled and followed for >24 months (median, 26.9; range, 24.0-29.5 months). The 2 groups had similar baseline clinical characteristics and similar success rates (93.8% [MD-ITI] vs 86.7% [LD-ITI]). Compared with LD-ITI, MD-ITI patients took a shorter median time to success (4.2 months vs 10.1 months) and partial success (2.7 months vs 6.6 months) and had lower mean rates for all bleeding (0.38/mo vs 1.40/mo) and joint bleeding (0.11/mo vs 0.83/mo). Between the 2 groups, although the MD-ITI group had higher rFVIII consumption (12,775 vs 7680 IU/kg), their total medication costs to success were similar (3626.49 vs 3240.38 US$/kg). Conclusion: For SHA-HTI children, the success rate and cost for MD-ITI and LD-ITI regimens were similar. MD-ITI regimen would be a priority for regions with economic constraints, considering the shorter time to success, better bleeding control, and no increase in medication cost.http://www.sciencedirect.com/science/article/pii/S2475037924003340immune tolerance inductionintermediate doselow doserandomized clinical trialsevere hemophilia A
spellingShingle Zhengping Li
Zekun Li
Xiaoling Cheng
Heng Zhang
Can Yang
Qian Xu
Zhenping Chen
Yingzi Zhen
Gang Li
Guoqing Liu
Wanru Yao
Min Zhou
Jiao Jin
Jie Huang
Yongjun Fang
Liangzhi Xie
Man-Chiu Poon
Runhui Wu
Intermediate-dose immune tolerance induction outperforms with faster success, less bleeding, and no added cost in comparison with low dose: a multicenter randomized clinical trial
Research and Practice in Thrombosis and Haemostasis
immune tolerance induction
intermediate dose
low dose
randomized clinical trial
severe hemophilia A
title Intermediate-dose immune tolerance induction outperforms with faster success, less bleeding, and no added cost in comparison with low dose: a multicenter randomized clinical trial
title_full Intermediate-dose immune tolerance induction outperforms with faster success, less bleeding, and no added cost in comparison with low dose: a multicenter randomized clinical trial
title_fullStr Intermediate-dose immune tolerance induction outperforms with faster success, less bleeding, and no added cost in comparison with low dose: a multicenter randomized clinical trial
title_full_unstemmed Intermediate-dose immune tolerance induction outperforms with faster success, less bleeding, and no added cost in comparison with low dose: a multicenter randomized clinical trial
title_short Intermediate-dose immune tolerance induction outperforms with faster success, less bleeding, and no added cost in comparison with low dose: a multicenter randomized clinical trial
title_sort intermediate dose immune tolerance induction outperforms with faster success less bleeding and no added cost in comparison with low dose a multicenter randomized clinical trial
topic immune tolerance induction
intermediate dose
low dose
randomized clinical trial
severe hemophilia A
url http://www.sciencedirect.com/science/article/pii/S2475037924003340
work_keys_str_mv AT zhengpingli intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT zekunli intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT xiaolingcheng intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT hengzhang intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT canyang intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT qianxu intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT zhenpingchen intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT yingzizhen intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT gangli intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT guoqingliu intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT wanruyao intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT minzhou intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT jiaojin intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT jiehuang intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT yongjunfang intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT liangzhixie intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT manchiupoon intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial
AT runhuiwu intermediatedoseimmunetoleranceinductionoutperformswithfastersuccesslessbleedingandnoaddedcostincomparisonwithlowdoseamulticenterrandomizedclinicaltrial