International Proof of Concept Therapeutic Stratification Trial of Molecular Anomalies in Relapsed or Refractory HEMatological Malignancies in Children, Subprotocol C Ruxolitinib + Venetoclax + Dexamethasone + Cyclophosphamide and Cytarabine in Pediatric Patients With Relapsed or Refractory Hematological Malignancies

HEM-iSMART is a master protocol which investigates multiple investigational medicinal products in children, adolescents and young adults (AYA) with relapsed/refractory (R/R) ALL and LBL. Sub-protocol C is a phase I/II trial evaluating the safety and efficacy of ruxolitinib and venetoclax in combination with dexamethasone, cyclophosphamide and cytarabine in children and AYA with R/R ped ALL/LBL whose tumor present with alterations in the IL7R/JAK-STAT pathway.

开始日期2024-10-01

申办/合作机构

Princess Maxima Centre For Pediatric Oncology

NCT05751044 / Not yet recruiting临床1/2期IIT

International Proof of Concept Therapeutic Stratification Trial of Molecular Anomalies in Relapsed or Refractory HEMatological Malignancies in Children, Sub-protocol B Dasatinib + Venetoclax + Dexamethasone + Cyclophosphamide and Cytarabine in Pediatric Patients With Relapsed or Refractory Hematological Malignancies

HEM-iSMART is a master protocol which investigates multiple investigational medicinal products in children, adolescents and young adults (AYA) with relapsed/refractory (R/R) ALL and LBL. Sub-protocol B is a phase I/II trial evaluating the safety and efficacy of dasatinib + venetocolax in combination with dexamethasone + Cyclophosphamide and cytarabine in children and AYA with R/R ped ALL/LBL whose tumor present with alterations in the MAPK/SRC pathway.

开始日期2024-10-01

申办/合作机构

Princess Maxima Centre For Pediatric Oncology

NCT05658640 / Recruiting临床1/2期IIT

International Proof of Concept Therapeutic Stratification Trial of Molecular Anomalies in Relapsed or Refractory HEMatological Malignancies in Children, Subprotocol D: Trametinib + Dexamethasone + Cyclophosphamide and Cytarabine in Pediatric Patients With Relapsed or Refractory Hematological Malignancies

HEM-iSMART is a master protocol which investigates multiple investigational medicinal products in children, adolescents and young adults (AYA) with relapsed/refractory (R/R) ALL and LBL. Sub-protocol D is a phase I/II trial evaluating the safety and efficacy of trametinib in combination with dexamethasone, cyclophosphamide and cytarabine in children and AYA with R/R ped ALL/LBL whose tumor present with alterations in the RAS-RAF-MAPK pathway.

开始日期2023-11-14

申办/合作机构

Princess Maxima Centre For Pediatric Oncology

100 项与难治性T淋巴细胞淋巴瘤相关的临床结果

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100 项与难治性T淋巴细胞淋巴瘤相关的转化医学

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0 项与难治性T淋巴细胞淋巴瘤相关的专利（医药）

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项与难治性T淋巴细胞淋巴瘤相关的文献（医药）

2024-02-01·European Journal of Haematology

Allogeneic hematopoetic stem cell transplant for patients with refractory T‐Cell lymphomas

Article

作者: Klyuchnikov, Evgeny ; Naim, Hassan ; Wolschke, Christine ; Ayuk, Francis ; Massoud, Radwan ; Janson, Dietlinde ; Kröger, Nicolaus

AbstractObjectiveAllogeneic stem cell transplantation (allo‐SCT) may have a curative potential due to the graft versus lymphoma effect. In this study, we aimed to compare transplant outcomes between refractory‐T‐NHL (ref‐NHL) and Chemosensitive‐T‐NHL (CS‐T‐NHL).Materials and MethodsWe retrospectively reviewed the records of 26 ref‐NHL and 29 CS‐T‐NHL consecutive patients who underwent allo‐SCT at our center and compared the transplant outcomes between the groups.ResultsAll patients were heavily pretreated with 27% of patients relapsing post‐auto‐SCT and two patients in the ref‐T‐NHL post‐allo‐SCT. Patients were transplanted mainly from unrelated donors. There were no differences in leucocytes and platelet engraftment between the two groups. At 3 years, the relapse incidence was 34% in Ref‐TNHL and 19% in CS‐TNHL (p = .33), with non‐relapse mortality rates of 28% and 22%, respectively (p = .52). Female patients and those with a previous auto‐SCT had lower relapse incidence (p = .045, p = .003). The 3‐year overall survival was 39% in Ref‐TNHL and 56% in CS‐TNHL (p = .15). Trends for improved progression‐free survival (PFS) and graft‐versus‐host disease relapse‐free survival (GRFS) were observed in the CS‐TNHL group (PFS: 60% vs. 30%, p = .075; GRFS: 38% vs. 21%, p = .1).ConclusionAcknowledging the retrospective nature of our study, our results indicate that allo‐SCT has a curative potential in patients with T‐NHL even in refractory status.

2023-06-22·Blood

How I treat newly diagnosed and refractory T-cell acute lymphoblastic lymphoma in children and young adults.

Article

作者: Ford, James B ; Si Lim, Stephanie J ; Hermiston, Michelle L

T-cell lymphoblastic lymphoma (T-LLy) and T-cell acute lymphoblastic leukemia (T-ALL) have historically been considered a spectrum of the same disease. However, recent evidence demonstrating differential responses to chemotherapy raise the possibility that T-LLy and T-ALL are distinct clinical and biologic entities. Here, we examine differences between the 2 diseases and use illustrative cases to highlight key recommendations on how to best treat patients with newly diagnosed and relapsed/refractory T-LLy. We discuss results of recent clinical trials incorporating use of nelarabine and bortezomib, choice of induction steroid, role of cranial radiotherapy, and risk stratification markers to identify patients at highest risk of relapse and to further refine current treatment strategies. Because prognosis for relapsed or refractory T-LLy patients is poor, we discuss ongoing investigations incorporating novel therapies, including immunotherapeutics, into upfront and salvage regimens and the role of hematopoietic stem cell transplantation.

2022-04-01·Asia-Pacific Journal of Clinical Oncology4区 · 医学

Dismal outcome of relapsed or primary refractory adult T‐cell lymphoblastic lymphoma: A retrospective study from China

4区 · 医学

Article

作者: Yang, Jianliang ; Chen, Haizhu ; Zhang, Changgong ; Zhou, Shengyu ; Sun, Yan ; Qin, Yan ; Yang, Sheng ; Gui, Lin ; Shi, Yuankai ; Liu, Peng ; He, Xiaohui

AbstractAimLittle is known about the outcome and prognostic factors of relapsed or refractory T‐cell lymphoblastic lymphoma (T‐LBL), especially in adult patients. The aim of this study was to analyze the characteristics, outcome and prognostic factors for this patient population.MethodsBetween January 2006 and December 2017, we retrospectively analyzed 84 adult patients with T‐LBL, and 44 relapsed or primary refractory patients were included in this analysis. Clinical features, treatment and follow‐up information were collected.ResultsFor all 44 patients, the median time to disease progression or relapse was 9.5 months after diagnosis. At a median follow‐up of 19.6 months, 40 (90.9%) patients died. The 3‐ and 5‐year overall survival (OS) rates after disease progression or relapse were 7.8% and 5.2%, respectively. Among 30 patients who had detailed information of second‐line treatment, only eight achieved a second complete remission (CR). Two of these eight patients subsequently underwent autologous or allogeneic stem cell transplantation (SCT), of whom one died from disease progression after autologous SCT, and one was free of event at 84 months after allogeneic SCT. Three of the rest six patients in second CR were still alive after chemotherapy alone. All the remaining patients who failed to gain second CR eventually died. In univariate analysis, only the achievement of second CR was positively predictive of OS (hazard ratio (HR) = 0.307; 95% confidence interval (CI), 0.104–0.905; P = 0.032).ConclusionThe outcome of adult patients with relapsed or refractory T‐LBL is extremely dismal. Patients with relapsed or refractory T‐LBL should be encouraged to participate in clinical trials where novel therapeutic approaches are available.