Clinical trial NCT03971318: A Prospective Multicenters Clinical Cohort Study of Stratified Treatment of Chinese Children With LBL
Official title: A Prospective Multicenter Cohort Study on the Efficacy and Safety of Stratified Treatment of Chinese Children With Lymphoblastic Lymphoma Based on Risk Factors
Study type: OBSERVATIONAL
Overall status: UNKNOWN
Conditions: Lymphoma, Non-Hodgkin
Lymphoblastic Lymphoma, Childhood
PTEN Loss
NOTCH1 Gene Mutation
Pediatric Cancer
Lead sponsor: Sun Yat-sen University
Sponsor class: OTHER
First posted: 2019-06-03
Enrollment: 300
Sex: ALL
Minimum age (years): 1
Maximum age (years): 18
Standard ages: CHILD
ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: lymphoblastic lymphoma patients#age at diagnosis \< 18 years old

Brief summary: With the development of molecular biology and precise medical treatment, new challenges have been raised in the diagnosis and treatment of non-Hodgkin lymphoma (NHL) in children. In recent years, the criteria for clinical staging and efficacy evaluation of NHL in children have been updated. Recent clinical studies of COG in the United States and LMB in France have confirmed that molecular biological markers such as Notch1, PTEN and LOH6q are significantly associated with the prognosis of T-lymphoblastic lymphoma (T-LBL). These molecular biological markers should be included in the new risk stratification system. High-intensity treatment of high-risk patients will improve survival. Recent studies have also suggested that PET/CT is helpful in evaluating residual lesions in patients with lymphoma after chemotherapy. In order to keep pace with the times in the diagnosis, clinical staging, risk stratification, efficacy evaluation and treatment of NHL in children. SCCCG-LBL-2017 was formulated by South China Children's Cancer Group of Non-Hodgkin lymphoma, which mainly updated in clinical staging, efficacy evaluation, risk stratification, treatment,etc..

Detailed description: Research purpose:

1. To investigate the efficacy and safety of SCCCG-LBL-2017 in Chinese children with LBL.
2. To explore the feasibility of risk stratification of T-LBL by combining genotyping.
3. To investigate the correlation between MDD and MRD in lymphoblastic lymphoma and prognosis.
4. To investigate the role of PET/CT in the assessment of residual lymphoblastic lymphoma.
5. To explore the effect of reducing HD-MTX dosage and shortening maintenance therapy time on the efficacy and survival of low-risk LBL patients.
6. To explore the effect of prolonging the duration of maintenance therapy on the efficacy and survival of high-risk LBL patients.

Eligibility criteria: Inclusion Criteria:

1. Age \< 18 years old
2. Pathologically confirmed lymphoblastic lymphoma
3. Newly diagnosed patients
4. Informed consent of guardian of children patients -

Exclusion Criteria:

1. Age \> 18 years old
2. Recurrent lymphoblastic lymphoma
3. Secondary immunodeficiency.

Primary outcomes:
measure: Event-free survival (EFS)
timeFrame: through study completion, maximal eight years
Secondary outcomes:
measure: Overall survival (OS)
timeFrame: through study completion, maximal eight years
measure: Relapse-free survival (RFS)
timeFrame: through study completion, maximal eight years
measure: Response rate (RR)
timeFrame: on an average 3 weeks after finish of treatment
measure: Adverse event rate
timeFrame: through study completion, maximal eight years
Other outcomes:
Locations:
city: Guangzhou
country: China
facility: Sun Yat-sen University Cancer Center
state: Guangdong
Study officials:
affiliation: Sun Yat-sen University
name: Zhen zijun
role: STUDY_DIRECTOR
References:
citation: Bonn BR, Rohde M, Zimmermann M, Krieger D, Oschlies I, Niggli F, Wrobel G, Attarbaschi A, Escherich G, Klapper W, Reiter A, Burkhardt B. Incidence and prognostic relevance of genetic variations in T-cell lymphoblastic lymphoma in childhood and adolescence. Blood. 2013 Apr 18;121(16):3153-60. doi: 10.1182/blood-2012-12-474148. Epub 2013 Feb 8.
citation: Callens C, Baleydier F, Lengline E, Ben Abdelali R, Petit A, Villarese P, Cieslak A, Minard-Colin V, Rullier A, Moreau A, Baruchel A, Schmitt C, Asnafi V, Bertrand Y, Macintyre E. Clinical impact of NOTCH1 and/or FBXW7 mutations, FLASH deletion, and TCR status in pediatric T-cell lymphoblastic lymphoma. J Clin Oncol. 2012 Jun 1;30(16):1966-73. doi: 10.1200/JCO.2011.39.7661. Epub 2012 Apr 30.