This is a phase II clinical trial using risk-adapted therapy. The treatment is acute lymphoblastic leukemia (ALL)-based therapy, using multi-agent regimens comprising of induction, consolidation, and continuation (maintenance) phases delivered over 24-30 months. Participants will be classified into 3 treatment stratums, based on bone marrow/peripheral blood lymphoma cells involvement at diagnosis and day 8 for T-lymphoblastic lymphoma and bone marrow/peripheral blood lymphoma cells involvement at diagnosis for B-lymphoblastic lymphoma. The Primary Objective of this study is: To improve the outcome of children with lymphoblastic lymphoma (LL) who have minimal disseminated disease (MDD) equal to or more than 1% at diagnosis by using MDD- and minimal residual disease (MRD)- based risk-adapted therapy. The Secondary Objectives of this study are: * To estimate the event-free survival and overall survival of children with lymphoblastic lymphoma who are treated with MDD- or MRD-based risk- directed therapy. * To evaluate the prognostic value of levels of MDD at diagnosis and MRD on day 8 of remission induction.
TREATMENT PLAN Treatment will consist of 3 main phases: remission induction, consolidation \[only for patients with any central nervous system (CNS) disease and/or testicular involvement\], and continuation. * Induction (6-7 weeks). * Consolidation for participants with CNS involvement or those with testicular disease only (10 weeks). * Reintensification - Participants with residual disease any time after induction therapy may receive 1-2 cycles of re-intensification therapy and may proceed to allogeneic stem cell transplant if suitable donor is available. * Continuation Therapy (98-120 weeks). * Intrathecal Chemotherapy (days 1 and 15; if needed also on days 8 and 22) TREATMENT SCHEME T lymphoblastic lymphoma: bone marrow/peripheral blood (BM/PB) involvement (MDD/MRD): Diagnosis: less than 1%; Day 8: +/- (Stratum 1) * Induction * Single dose of Cyclophosphamide * Steroid: prednisone * Continuation: 98 weeks T lymphoblastic lymphoma: BM/PB involvement (MDD/MRD): Diagnosis: equal to or greater than 1%; Day 8: - (Stratum 2) * Induction * Fractionated Cyclophosphamide * Steroid: prednisone * Continuation : 98 weeks T lymphoblastic lymphoma: BM/PB involvement (MDD/MRD): Diagnosis: equal to or greater than 1%; Day 8: + (Stratum 3) * Induction * Fractionated Cyclophosphamide * Steroid: prednisone and dexamethasone * Continuation: 120 weeks B lymphoblastic lymphoma: Stage I-III (Stratum 1) * Induction * Single dose of Cyclophosphamide * Steroid: prednisone * Continuation: 98 weeks B lymphoblastic lymphoma: Stage IV or testicular (Stratum 2) * Induction * Fractionated Cyclophosphamide * Steroid: prednisone * Continuation: 98 weeks Patients with CNS or testicular involvement will receive Consolidation therapy prior to continuation therapy and receive extended maintenance therapy (120 weeks). Any patient with detectable disease (MRD, bone marrow or biopsy of residual mass) at the end of induction may be considered for reintensification and/or hematopoietic stem cell transplantation (HSCT).
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
23
Given orally (PO).
Given intravenously (IV).
Given IV.
Given intramuscularly (IM) or IV.
Given IM or IV if allergy occurs with the first or second PEG-asparaginase dose.
Given IV.
Given IV.
Given IV or IT.
Given PO.
Given IV.
Given IV, IM or IT.
Given PO.
Given PO or IV.
Given IT.
Given IV.
Rady Children's Hospital San Diego
San Diego, California, United States
St. Jude Children's Research Hospital
Memphis, Tennessee, United States
Probability of Event-free Survival (EFS)
For EFS, relapse and second malignancies are considered as failures in addition to death in complete remission. The time to EFS will be set to 0 for patients who fail to achieve complete remission. Kaplan-Meier estimates of the OS and EFS curves are computed, along with estimates of standard errors by Peto's method. Please note the unit of measurement of probabilities are percentages.
Time frame: Two years post therapy.
Probability of Overall Survival (OS)
For OS, only deaths are considered failures for OS. Kaplan-Meier estimates of the OS curves are computed along with estimates of standard errors by Peto's method. Please note the unit of measurement of probabilities are percentages.
Time frame: Two years post therapy.
Minimal Disseminated Disease (MDD)
Detectable disease in bone marrow or blood: A binary measure, positive (detectable), negative (non-detectable)
Time frame: At Diagnosis
Minimal Residual Disease (MRD)
Detectable disease in bone marrow or blood: A binary measure, positive (detectable), negative (non-detectable)
Time frame: Day 8
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