Dataset Information

Core-binding factor acute myeloid leukemia with t(8;21): Risk factors and a novel scoring system (I-CBFit).

ABSTRACT:

Background

Although the prognosis of core-binding factor (CBF) acute myeloid leukemia (AML) is better than other subtypes of AML, 30% of patients still relapse and may require allogeneic hematopoietic cell transplantation (alloHCT). However, there is no validated widely accepted scoring system to predict patient subsets with higher risk of relapse.

Methods

Eleven centers in the US and Europe evaluated 247 patients with t(8;21)(q22;q22).

Results

Complete remission (CR) rate was high (92.7%), yet relapse occurred in 27.1% of patients. A total of 24.7% of patients received alloHCT. The median disease-free (DFS) and overall (OS) survival were 20.8 and 31.2 months, respectively. Age, KIT D816V mutated (11.3%) or nontested (36.4%) compared with KIT D816V wild type (52.5%), high white blood cell counts (WBC), and pseudodiploidy compared with hyper- or hypodiploidy were included in a scoring system (named I-CBFit). DFS rate at 2 years was 76% for patients with a low-risk I-CBFit score compared with 36% for those with a high-risk I-CBFit score (P < 0.0001). Low- vs high-risk OS at 2 years was 89% vs 51% (P < 0.0001).

Conclusions

I-CBFit composed of readily available risk factors can be useful to tailor the therapy of patients, especially for whom alloHCT is not need in CR1 (ie, patients with a low-risk I-CBFit score).

SUBMITTER: Ustun C

PROVIDER: S-EPMC6144246 | biostudies-literature | 2018 Sep

REPOSITORIES: biostudies-literature

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Publications

Core-binding factor acute myeloid leukemia with t(8;21): Risk factors and a novel scoring system (I-CBFit).

Ustun Celalettin C Morgan Elizabeth E Moodie Erica E M EEM Pullarkat Sheeja S Yeung Cecilia C Broesby-Olsen Sigurd S Ohgami Robert R Kim Young Y Sperr Wolfgang W Vestergaard Hanne H Chen Dong D Kluin Philip M PM Dolan Michelle M Mrózek Krzysztof K Czuchlewski David D Horny Hans-Peter HP George Tracy I TI Kristensen Thomas Kielsgaard TK Ku Nam K NK Yi Cecilia Arana CA Møller Michael Boe MB Marcucci Guido G Baughn Linda L Schiefer Ana-Iris AI Hilberink J R JR Pullarkat Vinod V Shanley Ryan R Kohlschmidt Jessica J Coulombe Janie J Salhotra Amandeep A Soma Lori L Cho Christina C Linden Michael A MA Akin Cem C Gotlib Jason J Hoermann Gregor G Hornick Jason J Nakamura Ryo R Deeg Joachim J Bloomfield Clara D CD Weisdorf Daniel D Litzow Mark R MR Valent Peter P Huls Gerwin G Perales Miguel-Angel MA Borthakur Gautam G

Cancer medicine 20180816 9

<h4>Background</h4>Although the prognosis of core-binding factor (CBF) acute myeloid leukemia (AML) is better than other subtypes of AML, 30% of patients still relapse and may require allogeneic hematopoietic cell transplantation (alloHCT). However, there is no validated widely accepted scoring system to predict patient subsets with higher risk of relapse.<h4>Methods</h4>Eleven centers in the US and Europe evaluated 247 patients with t(8;21)(q22;q22).<h4>Results</h4>Complete remission (CR) rate ...[more]

PMID: 30117318

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Project description:BackgroundPosttherapy measurable residual disease (MRD) positivity in core binding factor acute myeloid leukemia (CBF-AML) is associated with shorter relapse-free survival (RFS). Elimination of MRD measured via quantitative reverse transcription polymerase chain reaction (qRTPCR) for disease specific transcripts can potentially lead to better outcomes in CBF-AML.MethodsWe prospectively monitored the MRD using qRTPCR and flow cytometry on bone marrow samples in patients with newly diagnosed CBF-AML who received decitabine (DAC) maintenance therapy after fludarabine/cytarabine/G-CSF (FLAG)-based induction/consolidation regimen. Negative qRTPCR (CMR) was defined as fusion transcript <0.01%.ResultsThirty-one patients with CBF-AML including 14 with t(8;21) and 17 with inv(16) received parenteral DAC as maintenance therapy. Fifteen patients (48.3%) had completed FLAG-based induction/consolidation but with positive MRD (0.35%, range = 0.01%-0.91%) (Group 1). Sixteen patients (51.7%) could not complete recommended consolidations with FLAG-based regimen (due to older age or complications) and were switched to DAC maintenance (Group 2). In Group 2, eight patients (50%) had undetectable MRD (Group 2A) (all had qRTPCR ≤ 0.01%) and the other eight patients (50%) had residual fusion product by qRTPCR (0.1%, range = 0.02%-0.36%) (Group 2B) prior to starting DAC. Amongst the 23 patients who had a PCR ≥ 0.01% before maintenance therapy (Groups 1 and 2B), 12 patients (52%) attained a CMR as their best response (responders). The median pre-DAC qRTPCR amongst responders were 0.03% compared to 0.14% in nonresponders (p = .002). The median estimated molecular RFS amongst responders were 93.9 months. At a median follow-up of 59.3 months (13.2-106 months) from DAC initiation, 16 patients (51.6%) had to be initiated on a second line of therapy (40%, 25%, and 100% patients, respectively, in Groups1, 2A, and 2B). The median estimated time to new treatment between responders was 112.4 versus 5.8 months in nonresponders (hazard ratio = 0.16, 95% confidence interval = 0.04-0.54); however, there were no difference in overall survival between these groups (p = .37).ConclusionDAC is an effective maintenance therapy for CBF-AML patients with persistent fusion transcript at a low level after FLAG-based regimen. Attainment of CMR with DAC maintenance can lead to long-term remission in patients who have persistent MRD positive after FLAG-based regimen or are unable to receive the full course of consolidation therapy.

Dataset Information

Core-binding factor acute myeloid leukemia with t(8;21): Risk factors and a novel scoring system (I-CBFit).

Background

Methods

Results

Conclusions

Publications

Core-binding factor acute myeloid leukemia with t(8;21): Risk factors and a novel scoring system (I-CBFit).

Similar Datasets

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