Three of these 4 cases were found to contain rearrangements involving MECOM (chromosome 3) and noncoding regions of chromosome 2 (n=2, or chromosome 17 (n=1, Both ZFP36L2 and MSI2 are transcription factors expressed in hematopoietic cells.Ĭonclusion: Ras/MAPK pathway mutations and KMT2A rearrangements are frequent in pediatric tMN as are high levels of MECOM expression, a portion of which is driven by enhancer hijacking. The remaining 4 MECOM-High cases demonstrated allele-specific MECOM expression, suggesting a cis-regulatory element is driving this expression.

In addition to the RUNX1-MECOM event, these 24 MECOM-High cases included 18 with KMT2Ar (64% of KMT2Ar group) and 1 with a NUP98 fusion (NUP98-HHEX). Objectives: AALL0932 was designed to optimize maintenance therapy by asking: (1) whether giving vincristine/dexamethasone (VCR/DEX) pulses every 12 weeks was non-inferior to every 4 weeks and (2) whether a starting weekly oral methotrexate (MTX dose) of 40 mg/m²/dose would be superior to the standard 20 mg/m2/dose.ĭesign/Method: Average risk (AR) patients participating in the randomization included NCI SR B-ALL without CNS3 or testicular leukemia, unfavorable genetic characteristics or Down syndrome, AND either favorable genetics (trisomies of chromosomes 4 &10 or ETV6/RUNX1 fusion) with Day 8 peripheral blood (PB) minimal residual disease (MRD) ≥ 0.01% or CNS2 status, Day 29 bone marrow (BM) MRD 5 (MECOM-High).

There was thus uncertainty regarding the optimal frequency of pulses in COG ALL therapy.

showed that pulses of vincristine and dexamethasone during the continuation phase of BFM treatment do not affect overall survival in children with intermediate-risk ALL. Plenary Paper # 2001|||REDUCED FREQUENCY OF MAINTENANCE PULSES IN STANDARD-RISK B ACUTE LYMPHOBLASTIC LEUKEMIA AALL0932Īnne Angiolillo, Reuven Schore, John Kairalla, Meenakshi Devidas, Karen Rabin, Patrick Zweidler-McKay, Michael Borowitz, Brent Wood, Andrew Carroll, Nyla Heerema, Mary Relling, Ashley Lane, Kelly Maloney, Cindy Wang, William Carroll, Naomi Winick, Raetz Raetz, Mignon Loh, Stephen HungerĬhildren's National Medical Center, Washington, District of Columbia, United Statesīackground: A key component of COG maintenance chemotherapy for B-ALL has been vincristine/steroid pulses administered every 4 weeks.

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