Recruiting
Study Start: August, 2019
Study Completion: November, 2027
NCT04043494

International Cooperative Treatment Protocol for Children and Adolescents With Lymphoblastic Lymphoma (LBL 2018)

Keywords:
Cancer
Lymphoma

Brief Summary

Primary objectives:
  • Randomization R1, all patients eligible: To examine, whether the cumulative incidence of relapses with involvement of the CNS (CNS relapse, pCICR) can be decreased by a modified induction therapy including dexamethasone (experimental arm) instead of prednisone (standard arm)
  • Randomization R2, only patients with high risk LBL eligible to examine, whether the probability of event-free survival (pEFS) in these patients can be improved by receiving an intensified treatment arm versus a standard treatment arm (as used in the EURO-LB 02)

Detailed Description

The trial LBL 2018 is a collaborative prospective, multi-national, multi-center, randomized clinical trial for the treatment of children and adolescents with newly diagnosed lymphoblastic lymphoma.
The LBL 2018 trial will be open for the qualified centers of following participating study Groups (core study cohort): AIEOP (Italy), BFM (Austria, Czech Republic, Germany, Switzerland), BSPHO (Belgium), CoALL (Germany), DCOG (The Netherlands), NOPHO (Denmark, Finland, Norway, Sweden), PPLLSG (Poland), SEHOP (Spain) and SFCE (France). HKPHOSG (Hong Kong), HPOG (Hungary), ISPHO (Israel), NSPHO (Moscow), SHOP (Portugal) and SPS (Slovak Republic) start patient recruitment into the extended study cohort (without randomization). Over the trial period study groups may switch from the extended study cohort to the core study cohort.
Primary objectives:
  • Randomization R1, all patients eligible: To examine, whether the cumulative incidence of relapses with involvement of the CNS (CNS relapse, pCICR) can be decreased by a modified induction therapy including dexamethasone (experimental arm) instead of prednisone (standard arm)
  • Randomization R2, only patients with high risk LBL eligible to examine, whether the probability of event-free survival (pEFS) in these patients can be improved by receiving an intensified treatment arm versus a standard treatment arm (as used in the EURO-LB 02)
Patients are stratified into 3 different risk groups according to CNS status, immunophenotype, genetic markers and stage of disease at diagnosis: high risk group (HR), standard risk group I/II (SR I/II) and standard risk group (SR).
Patients in the risk groups SR I/II and SR are randomized (R1) in two arms after a cytoreductive prephase with prednisone. Patients in standard arm receive the standard induction phase with prednisone. Patients in the experimental arm receive an induction phase with dexamethasone instead of prednisone.
In SR group, induction phase is followed by the consolidation phase, the non-HR extra-compartment phase with HD-MTX (high-dose methotrexate), the reintensification phase and the maintenance therapy for the total therapy duration of 24 months. In SR I/II group, patients receive no reintensification phase. The induction phase is followed by the consolidation phase, the non-HR extra-compartment phase and the maintenance therapy for the total therapy duration of 24 months.
Patients in the HR group are eligible for randomization (R1) as outlined above. In addition high risk patients are eligible for second randomization (R2) at the end of induction phase. In the standard arm, HR-patients receive the consolidation phase and the non-HR extra-compartment phase. In the experimental arm, HR-patients receive a consolidation phase including two additional doses of PEG asparaginase and the HR-intensified extra-compartment phase consisting of two high risk courses alternating with two HD-MTX courses. Either phase is followed by the reintensification phase and the maintenance therapy for the total therapy duration of 24 months.
Patients with involvement of the CNS (CNS positive) are stratified to the high risk group (HR) and are eligible for both randomizations (R1 and R2). Additionally, patients with CNS involvement (CNS positive) receive intensified intrathecal therapy. Intrathecal therapy consists of TIT (triple intrathecal therapy) after diagnosis of CNS involvement. TIT is administered twice weekly until clearance of blasts in the cerebrospinal fluid is achieved. Further intrathecal therapy is provided at the same points in time as for patients without CNS involvement, but TIT instead of MTX IT. In addition, patients receive four additional doses of TIT during maintenance. Cranial irradiation is omitted for patients with CNS involvement.

Intervention / Treatment

01
Cyclophosphamide
Drug
02
Cytarabine
Drug
03
Dexamethasone
Drug
04
Daunorubicin
Drug
05
Doxorubicin
Drug
06
Ifosfamide
Drug
07
6-Mercaptopurine
Drug
08
Methotrexate
Drug
09
PEG asparaginase
Drug
10
Prednisone
Drug
11
Prednisolone
Drug
12
Thioguanine
Drug
13
Vincristine
Drug
14
Vindesine
Drug

Study Design

Primary Purpose

Treatment

Allocation

Randomized

Interventional Model

Parallel Assignment

Masking

None (Open Label)

Arm Groups

Other: SR I/II: R1 into protocol Ia-Pred

1. cytoreductive prephase with prednisone
2. standard induction phase (protocol Ia-prednisone)
3. consolidation phase (protocol Ib)
4. non-HR extra-compartment phase (protocol M)
5. maintenance therapy

Experimental: SR I/II: R1 into protocol Ia-Dexa

1. cytoreductive prephase with prednisone
2. experimental induction phase (protocol Ia-dexamethasone)
3. consolidation phase (protocol Ib)
4. non-HR extra-compartment Phase (protocol M)
5. maintenance therapy

Other: SR: R1 into protocol Ia-Pred

1. cytoreductive prephase with prednisone
2. standard induction phase (protocol Ia-prednisone)
3. consolidation phase (protocol Ib)
4. non-HR extra-compartment phase (protocol M)
5. reintensification phase (protocol II)
6. maintenance therapy

Experimental: SR: R1 into protocol Ia-Dexa

1. cytoreductive prephase with prednisone
2. experimental induction phase (protocol Ia-dexamethasone)
3. consolidation phase (protocol Ib)
4. non-HR extra-compartment phase (protocol M)
5. reintensification phase (protocol II)
6. maintenance therapy

Other: HR: R1 into "Pred" and R2 into non-HR extra-compartment phase

1. cytoreductive prephase with prednisone
2. standard induction phase (protocol Ia-prednisone)
3. consolidation phase (protocol Ib)
4. non-HR extra-compartment phase (protocol M)
5. reintensification phase (protocol II)
6. maintenance therapy

Experimental: HR: R1 into "Dexa" and R2 into non-HR extra-compartment phase

1. cytoreductive prephase with prednisone
2. experimental induction phase (protocol Ia-dexamethasone)
3. consolidation phase (protocol Ib)
4. non-HR extra-compartment phase (protocol M)
5. reintensification phase (protocol II)
6. maintenance therapy

Experimental: HR: R1 into "Pred" and R2 into HR extra-compartment phase

1. cytoreductive prephase with prednisone
2. standard induction phase (protocol Ia-prednisone)
3. consolidation phase (protocol Ib*)
4. HR extra-compartment phase (intensified protocol M)
5. reintensification phase (protocol II)
6. maintenance therapy

Experimental: HR: R1 into "Dexa" and R2 into HR extra-compartment phase

1. cytoreductive prephase with prednisone
2. experimental induction phase (protocol Ia-dexamethasone)
3. consolidation phase (protocol Ib*)
4. HR extra-compartment phase (intensified protocol M)
5. reintensification phase (protocol II)
6. maintenance therapy

Eligibility Criteria

  • newly diagnosed lymphoblastic lymphoma
  • age <18 years
  • patient enrolled in a participating center
  • written informed consent of patient (>14 years of age or according to local law and regulation) and parents to trial participation and transfer and processing of data
  • willingness of patients and the investigator/pathologist to provide adequate slides/blocks for reference (molecular) pathology and international pathology panel and/or fresh or fresh frozen samples for genetic risk group stratification if these samples are available after standard diagnostic procedures.

  • lymphoblastic lymphoma as secondary malignancy
  • non-lymphoma related relevant medical, psychiatric or social conditions incompatible with trial treatment, including among others

    • prior organ transplant
    • severe immunodeficiency
    • demyelinating Charcot-Marie Tooth syndrome
    • serious acute or chronic infections, such as HIV, VZV and tuberculosis
    • urinary tract infection, cystitis, urinary outflow obstruction, severe renal impairment (creatinine clearance less than 20 ml/min)
    • severe hepatic impairment (bilirubin >3 times ULN, transaminases >10 times ULN)
    • myocardial insufficiency, severe arrhythmias
    • ulcers of the oral cavity and known active gastrointestinal ulcer disease
    • known hypersensitivity to any IMP and to any excipient (listed in section 6.1 of the respective SmPC)

  • steroid pre-treatment with ≥ 1 mg/kg/d for more than two weeks during the last month before diagnosis
  • vaccination with live vaccines within 2 weeks before start of protocol treatment
  • treatment started according to another protocol or pre-treatment with cytostatic drugs
  • participation in another clinical trial that interferes with the protocol, except NHL-BFM Registry 2012 and trials with different endpoints, involving aspects of supportive treatment, which can run parallel to LBL 2018 without influencing the outcome of this trial (e.g. trials on antiemetics, antibiotics, strategies for psychosocial support)
  • evidence of pregnancy or lactation period
  • sexually active adolescents not willing to use highly effective contraceptive method (pearl index < 1) until 12 months after end of cytostatic therapy

Outcome Measures

Primary Outcome Measures

Cumulative incidence of relapse with involvement of the CNS (CNS-relapse, pCICR)

The time to relapse is defined as the time from randomization to the first relapse or the date of last follow-up. Other events (non-response, progressive disease, relapse, second malignancy or death before and in CR) will be taken into account as competing events.

Through study completion, maximal 7.25 years

Estimated probability of event-free survival (pEFS)

The pEFS is defined as the time from randomization to the first event (non-response, progressive disease, relapse, second malignancy or death from any cause) or date of last follow-up.

Through study completion, maximal 7.25 years

Secondary Outcome Measures

Survival (pOS)

Survival (pOS) is defined as time from diagnosis to death due to any cause or to the date of last contact for patients alive

Through study completion, maximal 7.25 years

Frequency of treatment-related toxicity overall and in specific protocol elements, randomized arms and during follow up

Through study completion, maximal 7.25 years

Frequency of treatment-related mortality overall and in specific protocol elements, randomized arms and during follow up

Through study completion, maximal 7.25 years

Frequency of adverse events of interest and severe adverse events overall

Through study completion, maximal 7.25 years

Rate of evaluable patients for risk group stratification

During recruitment

Cumulative incidence of relapses in association with molecular markers

Through study completion, maximal 7.25 years

Cumulative incidence of relapses in association with minimal residual disease results

Through study completion, maximal 7.25 years

Where is this study conducted?

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