Year: 2024
Type: article
Study design: Observational Study
Abstract: The BCL6-corepressor (BCOR) is a tumor-suppressor gene located on the short arm of chromosome X. Data are limited regarding factors predicting survival in BCOR-mutated (mBCOR) acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS). We evaluated 138 patients with mBCOR myeloid disorders, of which 36 (26.1%) had AML and 63 (45.6%) had MDS. Sixty-six (47.8%) patients had a normal karyotype while 18 (13%) patients had complex karyotype. BCOR-mutated MDS/AML were highly associated with RUNX1 and U2AF1 co-mutations. In contrast, TP53 mutation was infrequently seen with mBCOR MDS. Patients with an isolated BCOR mutation had similar survival compared to those with high-risk co-mutations by European LeukemiaNet (ELN) 2022 criteria (median OS 1.16 vs. 1.27 years, P=0.46). Complex karyotype adversely impacted survival among mBCOR AML/MDS (HR 4.12, P<0.001), while allogeneic stem cell transplant (alloSCT) improved survival (HR 0.38, P=0.04). However, RUNX1 co-mutation was associated with an increased risk of post-alloSCT relapse (HR 88.0, P=0.02), whereas melphalan-based conditioning was associated with a decreased relapse risk (HR 0.02, P=0.01). We conclude that mBCOR is a high-risk feature across MDS/AML, and that alloSCT improves survival in this population.
Source: Haematologica
Authors: Anmol Baranwal, Mark Gurney, Rami Basmaci, Bahga Katamesh, Rong He, David S. Viswanatha, Patricia Greipp, James Foran, Talha Badar, Hemant Murthy, Cecilia Arana Yi, Jeanne Palmer, Abhishek A. Mangaonkar, Mrinal M. Patnaik, Mark R. Litzow, William J. Hogan, Kebede Begna, Naseema Gangat, Ayalew Tefferi, Aref Al-Kali, Mithun V. Shah, Hassan B. Alkhateeb
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,157
Funders: Celgene, Astellas Pharma
OpenAlex ID: W4391438375