Table 2.

Summary of treatment results of recent clinical trials for children and adolescents with relapsed/refractory T-cell lymphoblastic lymphoma

TrialStudy yStudy populationNumber of patients with r/r/ T-LL (total number of r/r patients)TreatmentOutcomeOutcome w/ HSCT
CCG-591269  1991-1994 r/r HD/NHL 68 NHL (97) DECAL ± HSCT 23% ± 5% EFS; 31% ± 14% OS (all NHL, not just T-LL) No significant benefit to HSCT 
BFM 90 and 9558  1990-2003 T/B-LLy 28 (251) ALL-BFM ± HSCT 14% ± 7% OS 42% OS 
JPLSG13  1996-2004 T/B-LLy 32 (48) ALL-backbone ± HSCT 32.8% ± 8.6% OS HR 2.64 for absence of HSCT 
CIBMTR70  1990-2005 NHL 53 LL (182) Allo/Auto HSCT  40% EFS (allo); 4% EFS (auto) 
AIEOP-NHL-9771  1997-2007 T/B-LLy 21 LSA2-L2 ± HSCT OS 28% 41% OS (5/6 allo) 
EORTC 58951 and LMT 9672  1997-2008 T/B-LLy 18 (23) Various chemo regimens ± HSCT 8.7% EFS; 8.7% OS 20% OS 
AALL07P159  2009-2014 r/r T/B-ALL, LLy 10 (135) Bortezomib + 4-drug re-induction 70% ORR NA 
MD Anderson venetoclax study65   T/B-ALL, LLy 6 (18) Venetoclax + chemo ORR 83%, PFS 7.34 mo, OS 9.14 mo NA 
Delphinus (NCT03384654)62  2018-2022 r/r T-ALL, LLy 10 BFM + daratumumab 40% CR NA 
Case study64   r/r T-ALL, LLy 1 (2) Daratumumab + nelarabine Alive at 3.5 years s/p allo-HSCT NA 
Campus ALL study73  2018-2020 r/r T/B-ALL, LLy 1 (20) Daratumumab ± chemo Non-responder NA 
CD7 CAR T cell therapy68  2020-2021 r/r T-ALL, LLy NS7CAR T therapy 100% CRi on day 28 NA 
Phase 1 study of venetoclax + navitoclax + chemotherapy (NCT03181126) 2017-2019 r/r T-ALL, LLy 2 (47) Venetoclax + low-dose navitoclax + chemo 60% CR NA 
TrialStudy yStudy populationNumber of patients with r/r/ T-LL (total number of r/r patients)TreatmentOutcomeOutcome w/ HSCT
CCG-591269  1991-1994 r/r HD/NHL 68 NHL (97) DECAL ± HSCT 23% ± 5% EFS; 31% ± 14% OS (all NHL, not just T-LL) No significant benefit to HSCT 
BFM 90 and 9558  1990-2003 T/B-LLy 28 (251) ALL-BFM ± HSCT 14% ± 7% OS 42% OS 
JPLSG13  1996-2004 T/B-LLy 32 (48) ALL-backbone ± HSCT 32.8% ± 8.6% OS HR 2.64 for absence of HSCT 
CIBMTR70  1990-2005 NHL 53 LL (182) Allo/Auto HSCT  40% EFS (allo); 4% EFS (auto) 
AIEOP-NHL-9771  1997-2007 T/B-LLy 21 LSA2-L2 ± HSCT OS 28% 41% OS (5/6 allo) 
EORTC 58951 and LMT 9672  1997-2008 T/B-LLy 18 (23) Various chemo regimens ± HSCT 8.7% EFS; 8.7% OS 20% OS 
AALL07P159  2009-2014 r/r T/B-ALL, LLy 10 (135) Bortezomib + 4-drug re-induction 70% ORR NA 
MD Anderson venetoclax study65   T/B-ALL, LLy 6 (18) Venetoclax + chemo ORR 83%, PFS 7.34 mo, OS 9.14 mo NA 
Delphinus (NCT03384654)62  2018-2022 r/r T-ALL, LLy 10 BFM + daratumumab 40% CR NA 
Case study64   r/r T-ALL, LLy 1 (2) Daratumumab + nelarabine Alive at 3.5 years s/p allo-HSCT NA 
Campus ALL study73  2018-2020 r/r T/B-ALL, LLy 1 (20) Daratumumab ± chemo Non-responder NA 
CD7 CAR T cell therapy68  2020-2021 r/r T-ALL, LLy NS7CAR T therapy 100% CRi on day 28 NA 
Phase 1 study of venetoclax + navitoclax + chemotherapy (NCT03181126) 2017-2019 r/r T-ALL, LLy 2 (47) Venetoclax + low-dose navitoclax + chemo 60% CR NA 

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