3159 – Ph-Like and Ikzf1plus Features in Children With Down Syndrome Acute Lymphoblastic Leukaemia

Palmi, Chiara; Bresolin, Silvia; Junk, Stefanie V.; Fazio, Grazia; Silvestri, Daniela; Žaliová, Markéta; Stanulla, Martin; Moericke, Anja; Zimmermann, Martin; Schrappe, Martin; Buldini, Barbara; Saitta, Claudia; Galbiati, Marta; Bardini, Michela; Nigro, Luca Lo; Conter, Valentino; Valsecchi, Maria Grazia; Biondi, Andrea; Kronnie, Geertruy te; Cario, Gunnar; Cazzaniga, Giovanni

doi:10.1016/j.exphem.2022.07.215

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“…These patients may benefit from emerging therapeutic approaches, including immunotherapy or targeted therapies that specially address the altered signalling networks associated with IKZF1 abnormalities. 31,32…”

Section: Discussionmentioning

confidence: 99%

“…We further demonstrated that IKZF1 plus could offer enhanced prognostic value in the subgroup of Day 15 MRD‐positive provisional high‐risk patients, who were classified after excluding favourable features such as NCI standard risk, ETV6::RUNX1 fusion and high hyperdiploidy in the Ph‐negative B‐ALL. These patients may benefit from emerging therapeutic approaches, including immunotherapy or targeted therapies that specially address the altered signalling networks associated with IKZF1 abnormalities 31,32 …”

Section: Discussionmentioning

confidence: 99%

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Refining risk stratification in paediatric B‐acute lymphoblastic leukaemia: Combining IKZF1^plus and Day 15 MRD positivity

Liu,

Huang,

Jaing

et al. 2024

Br J Haematol

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SummaryThis study investigates the potential utility of IKZF1 deletion as an additional high‐risk marker for paediatric acute lymphoblastic leukaemia (ALL). The prognostic impact of IKZF1 status, in conjunction with minimal/measurable residual disease (MRD), was evaluated within the MRD‐guided TPOG‐ALL‐2013 protocol using 412 newly diagnosed B‐ALL patients aged 1–18. IKZF1 status was determined using multiplex ligation‐dependent probe amplification. IKZF1 deletions, when co‐occurring with CDKN2A, CDKN2B, PAX5 or PAR1 region deletions in the absence of ERG deletions, were termed IKZF1plus. Both IKZF1 deletion (14.6%) and IKZF1plus (7.8%) independently predicted poorer outcomes in B‐ALL. IKZF1plus was observed in 4.1% of Philadelphia‐negative ALL, with a significantly lower 5‐year event‐free survival (53.9%) compared to IKZF1 deletion alone (83.8%) and wild‐type IKZF1 (91.3%) (p < 0.0001). Among patients with Day 15 MRD ≥0.01%, provisional high‐risk patients with IKZF1plus exhibited the worst outcomes in event‐free survival (42.0%), relapse‐free survival (48.0%) and overall survival (72.7%) compared to other groups (p < 0.0001). Integration of IKZF1plus and positive Day 15 MRD identified a subgroup of Philadelphia‐negative B‐ALL with a 50% risk of relapse. This study highlights the importance of assessing IKZF1plus alongside Day 15 MRD positivity to identify patients at increased risk of adverse outcomes, potentially minimizing overtreatment.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Refining risk stratification in paediatric B‐acute lymphoblastic leukaemia: Combining IKZF1^plus and Day 15 MRD positivity

Liu,

Huang,

Jaing

et al. 2024

Br J Haematol

View full text Add to dashboard Cite

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3159 – Ph-Like and Ikzf1plus Features in Children With Down Syndrome Acute Lymphoblastic Leukaemia

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Refining risk stratification in paediatric B‐acute lymphoblastic leukaemia: Combining IKZF1^plus and Day 15 MRD positivity

Refining risk stratification in paediatric B‐acute lymphoblastic leukaemia: Combining IKZF1^plus and Day 15 MRD positivity

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3159 – Ph-Like and Ikzf1plus Features in Children With Down Syndrome Acute Lymphoblastic Leukaemia

Cited by 1 publication

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Refining risk stratification in paediatric B‐acute lymphoblastic leukaemia: Combining IKZF1plus and Day 15 MRD positivity

Refining risk stratification in paediatric B‐acute lymphoblastic leukaemia: Combining IKZF1plus and Day 15 MRD positivity

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Refining risk stratification in paediatric B‐acute lymphoblastic leukaemia: Combining IKZF1^plus and Day 15 MRD positivity

Refining risk stratification in paediatric B‐acute lymphoblastic leukaemia: Combining IKZF1^plus and Day 15 MRD positivity