Table 2 Overview of the demographic features, treatment response and outcome of patients with DUX4-rearranged ALL, and those exhibiting markers associated with DUX4-rearrnangement, from key studies.
From: DUX4-rearranged B-ALL: deciphering a biological and clinical conundrum
Study | Trial | Number of DUX4r cases | Frequency in BCP-ALLa | Median age (years) | MRD positive at EOI | Treated as standard risk | Treatment period | Outcome at 5 years |
|---|---|---|---|---|---|---|---|---|
Lilljebjorn et al. [52] | NOPHO-ALL 1992/2000/2008 | 28 | 4% | 9 | NA | NA | 1992–2013 | 4/28 relapsed |
Yasuda et al. [3] | ALL202-U | 10 | 14% | 19c | NA | NA | 2002–2009 | DFS > 80% |
Zhang et al. [2] | St Jude / COG | >50 | NA | NA | NA | NA | NA | EFS > 90% |
Li et al. [98] | Multiple cohorts | 63 | 5% | NA | NA | NA | NA | Paed OS > 90%; Adult >70% |
Schinnerl et al. [74] | AEIOP-BFM 2000/2009/2017 | 70 | 6% | 10 | 88% | 9% | 1999–2023 | EFS 89% (10 y) |
Ueno et al. [81] | JACLS-ALL02 & TCCSG-L04-16 | 57 | 6% | 9 | NA | NA | 2002–2013 | EFS 81% |
Jeha et al. [99] | St Jude Total Therapy 16 | 20 | 4% | 8 | 5%d | 40% | 2007–2017 | EFS 95% |
Li et al. [61] | MASpore 2003/2010 | 51 | 14% | 10 | 78% | NA | 2002–2011 | OS > 95% |
Paietta et al. [7] | ECOG2993 | 22 | 8%b | 26c | NA | NA | 1993–2005 | OS 75% |
Brady et al. [64] | Multiple COG & St Jude trials | 96 | 4% | 13 | NA | NA | NA | OS > 95% |
Leongamornlert et al. [57] | UKALL14 | 8 | NA | 40c | 13% | 25% | 2012–2017 | 5/8 relapsed |
Schwab et al. [6] | UKALL2003 | 80 | 5% | 9 | 55% | 36% | 2003–2011 | EFS 95% (10 y) |
Yu et al. [8] | TPOG-ALL 2002/2013 | 26 | 5%b | NA | 29% | 27% | 2002–2020 | EFS 83% |
Study | Trial | Number of cases showing surrogate marker | Frequency in BCP-ALLa | Median age (years) | MRD positive at EOI | Treated as standard risk | Treatment period | Outcome at 5 years |
Clappier et al. [62] | EORTC-CLG 58951 | 29 ERGdel | 3% | 7 | 100% | 0% | 1993–2006 | EFS > 86% (8 y) |
Zaliova et al. [60] | BFM-ALL-2000 | 60 ERGdel | 5% | NA | 60% | 11% | 2000–2006 | EFS 90% |
Buldini et al. [75] | AIEOP-BFM ALL 2009 | 160 CD371+ | 9% | 20% ≥10 y | 89% | 11% | 2014–2017 | EFS 88% |
