Genetic subtype-guided immunochemotherapy in relapsed and refractory diffuse large B cell lymphoma: a phase 2 investigator-initiated nonrandomized clinical trial (GUIDANCE-06)

Abstract Improving the outcome of relapsed or refractory diffuse large B-cell lymphoma (R/R DLBCL) remained an unmet need. The aim of this single-center, phase 2 trial was to evaluate the efficacy and safety of genetic subtype-guided immunochemotherapy (R-ICE-X) in patients with R/R DLBCL: R-ICE-zan...

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Main Authors: Yi-Ge Shen, Qing Shi, Wei Tang, Peng-Peng Xu, Yi-Wen Cao, Meng-Meng Ji, Zhong Zheng, Shu Cheng, Li Wang, Wei-Li Zhao
Format: Article
Language:English
Published: Nature Publishing Group 2025-07-01
Series:Signal Transduction and Targeted Therapy
Online Access:https://doi.org/10.1038/s41392-025-02316-6
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author Yi-Ge Shen
Qing Shi
Wei Tang
Peng-Peng Xu
Yi-Wen Cao
Meng-Meng Ji
Zhong Zheng
Shu Cheng
Li Wang
Wei-Li Zhao
author_facet Yi-Ge Shen
Qing Shi
Wei Tang
Peng-Peng Xu
Yi-Wen Cao
Meng-Meng Ji
Zhong Zheng
Shu Cheng
Li Wang
Wei-Li Zhao
author_sort Yi-Ge Shen
collection DOAJ
description Abstract Improving the outcome of relapsed or refractory diffuse large B-cell lymphoma (R/R DLBCL) remained an unmet need. The aim of this single-center, phase 2 trial was to evaluate the efficacy and safety of genetic subtype-guided immunochemotherapy (R-ICE-X) in patients with R/R DLBCL: R-ICE-zanubrutinib for MCD-like and BN2-like, R-ICE-lenalidomide for N1-like and NOS, R-ICE-decitabine for TP53 Mut, R-ICE-chidamide for EZB-like, and R-ICE-tofacitinib for ST2-like subtype. Enrolled patients were treated with assigned regimens for three cycles, and then responders were treated with autologous hematopoietic stem cell transplantation (ASCT) or 3 cycles of R-ICE-X consolidation and lenalidomide maintenance. The primary endpoint was the complete response (CR) rate. The secondary endpoints included overall response rate (ORR), progression-free survival (PFS), overall survival (OS), and safety assessment. Between April 26, 2022, and July 31, 2024, 76 patients were enrolled, with 74 adhering to and 2 deviating from the protocol. Among all, the CR rate was 56.6% (95% CI, 45.2–68.0%), and the ORR was 76.3% (95% CI, 66.5–86.1%) at the end of induction. With a median follow-up of 19.5 months, the 2-year PFS rate was 69.3% (95% CI, 56.6–79.0%), and the 2-year OS rate was 88.3% (95% CI, 77.6–94.0%). The primary grade 3-4 adverse events were neutropenia (30%) and thrombocytopenia (25%). The presence of bulky disease and CD70 mutation was linked to poor prognosis. Further gene set enrichment analysis revealed that up-regulated PI3K-AKT-mTOR signaling pathway and reduced immune cell infiltration were significantly associated with disease progression. Patients with mesenchymal or inflammatory lymphoma microenvironment subtypes benefited from R-ICE-X treatment. Our findings highlight the efficacy and safety of R-ICE-X, a mechanism-based tailored therapy, which dually targets genetic and microenvironmental alterations in R/R DLBCL.
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spelling doaj-art-7ca9dd6b80c14780bd706aca1a45f8f22025-08-20T03:43:37ZengNature Publishing GroupSignal Transduction and Targeted Therapy2059-36352025-07-0110111110.1038/s41392-025-02316-6Genetic subtype-guided immunochemotherapy in relapsed and refractory diffuse large B cell lymphoma: a phase 2 investigator-initiated nonrandomized clinical trial (GUIDANCE-06)Yi-Ge Shen0Qing Shi1Wei Tang2Peng-Peng Xu3Yi-Wen Cao4Meng-Meng Ji5Zhong Zheng6Shu Cheng7Li Wang8Wei-Li Zhao9Shanghai Institute of Hematology, State Key Laboratory of Medical Genomics; National Research Center for Translational Medicine at Shanghai, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai Institute of Hematology, State Key Laboratory of Medical Genomics; National Research Center for Translational Medicine at Shanghai, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai Institute of Hematology, State Key Laboratory of Medical Genomics; National Research Center for Translational Medicine at Shanghai, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai Institute of Hematology, State Key Laboratory of Medical Genomics; National Research Center for Translational Medicine at Shanghai, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai Institute of Hematology, State Key Laboratory of Medical Genomics; National Research Center for Translational Medicine at Shanghai, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai Institute of Hematology, State Key Laboratory of Medical Genomics; National Research Center for Translational Medicine at Shanghai, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai Institute of Hematology, State Key Laboratory of Medical Genomics; National Research Center for Translational Medicine at Shanghai, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai Institute of Hematology, State Key Laboratory of Medical Genomics; National Research Center for Translational Medicine at Shanghai, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai Institute of Hematology, State Key Laboratory of Medical Genomics; National Research Center for Translational Medicine at Shanghai, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai Institute of Hematology, State Key Laboratory of Medical Genomics; National Research Center for Translational Medicine at Shanghai, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineAbstract Improving the outcome of relapsed or refractory diffuse large B-cell lymphoma (R/R DLBCL) remained an unmet need. The aim of this single-center, phase 2 trial was to evaluate the efficacy and safety of genetic subtype-guided immunochemotherapy (R-ICE-X) in patients with R/R DLBCL: R-ICE-zanubrutinib for MCD-like and BN2-like, R-ICE-lenalidomide for N1-like and NOS, R-ICE-decitabine for TP53 Mut, R-ICE-chidamide for EZB-like, and R-ICE-tofacitinib for ST2-like subtype. Enrolled patients were treated with assigned regimens for three cycles, and then responders were treated with autologous hematopoietic stem cell transplantation (ASCT) or 3 cycles of R-ICE-X consolidation and lenalidomide maintenance. The primary endpoint was the complete response (CR) rate. The secondary endpoints included overall response rate (ORR), progression-free survival (PFS), overall survival (OS), and safety assessment. Between April 26, 2022, and July 31, 2024, 76 patients were enrolled, with 74 adhering to and 2 deviating from the protocol. Among all, the CR rate was 56.6% (95% CI, 45.2–68.0%), and the ORR was 76.3% (95% CI, 66.5–86.1%) at the end of induction. With a median follow-up of 19.5 months, the 2-year PFS rate was 69.3% (95% CI, 56.6–79.0%), and the 2-year OS rate was 88.3% (95% CI, 77.6–94.0%). The primary grade 3-4 adverse events were neutropenia (30%) and thrombocytopenia (25%). The presence of bulky disease and CD70 mutation was linked to poor prognosis. Further gene set enrichment analysis revealed that up-regulated PI3K-AKT-mTOR signaling pathway and reduced immune cell infiltration were significantly associated with disease progression. Patients with mesenchymal or inflammatory lymphoma microenvironment subtypes benefited from R-ICE-X treatment. Our findings highlight the efficacy and safety of R-ICE-X, a mechanism-based tailored therapy, which dually targets genetic and microenvironmental alterations in R/R DLBCL.https://doi.org/10.1038/s41392-025-02316-6
spellingShingle Yi-Ge Shen
Qing Shi
Wei Tang
Peng-Peng Xu
Yi-Wen Cao
Meng-Meng Ji
Zhong Zheng
Shu Cheng
Li Wang
Wei-Li Zhao
Genetic subtype-guided immunochemotherapy in relapsed and refractory diffuse large B cell lymphoma: a phase 2 investigator-initiated nonrandomized clinical trial (GUIDANCE-06)
Signal Transduction and Targeted Therapy
title Genetic subtype-guided immunochemotherapy in relapsed and refractory diffuse large B cell lymphoma: a phase 2 investigator-initiated nonrandomized clinical trial (GUIDANCE-06)
title_full Genetic subtype-guided immunochemotherapy in relapsed and refractory diffuse large B cell lymphoma: a phase 2 investigator-initiated nonrandomized clinical trial (GUIDANCE-06)
title_fullStr Genetic subtype-guided immunochemotherapy in relapsed and refractory diffuse large B cell lymphoma: a phase 2 investigator-initiated nonrandomized clinical trial (GUIDANCE-06)
title_full_unstemmed Genetic subtype-guided immunochemotherapy in relapsed and refractory diffuse large B cell lymphoma: a phase 2 investigator-initiated nonrandomized clinical trial (GUIDANCE-06)
title_short Genetic subtype-guided immunochemotherapy in relapsed and refractory diffuse large B cell lymphoma: a phase 2 investigator-initiated nonrandomized clinical trial (GUIDANCE-06)
title_sort genetic subtype guided immunochemotherapy in relapsed and refractory diffuse large b cell lymphoma a phase 2 investigator initiated nonrandomized clinical trial guidance 06
url https://doi.org/10.1038/s41392-025-02316-6
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