Abstract | BACKGROUND: PATIENTS AND METHODS: A retrospective chart review of patients with CML who had CBF rearrangement, t(8;21) or inv(16), in Philadelphia chromosome-positive clones was conducted. Additional cases of CML with CBF rearrangements were identified through literature review. RESULTS: Between August 1997 and December 2014, we identified 11 patients who had Philadelphia chromosome and CBF rearrangement in the same clones: 1 (9%) with t(8;21) and 10 (91%) with inv(16). Nine (82%) patients were in blast phase, and 2 (18%) in second chronic phase. Four (36%) patients received tyrosine kinase inhibitor monotherapy, 2 (18%) received tyrosine kinase inhibitor and chemotherapy, and 5 (45%) received chemotherapy only. Three (27%) patients achieved complete remission with incomplete count recovery, and 4 (36%) had no response after the initial therapy. Three (27%) patients underwent allogeneic stem cell transplantation. The median event-free survival and overall survival for the 11 patients were 2 months and 6 months, respectively. Literature review identified 14 patients with CML with CBF rearrangement with a median overall survival of 14 months. CONCLUSION: Acquisition of CBF rearrangement in addition to Philadelphia chromosome is a rare phenomenon associated with poor prognosis. CBF rearrangements as ACAs in patients with CML can be considered high-risk features.
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Authors | Kiyomi Morita, Elias Jabbour, Farhad Ravandi, Gautam Borthakur, Joseph D Khoury, Shimin Hu, Guillermo Garcia-Manero, William Wierda, Ghayas Issa, Naval Daver, Naveen Pemmaraju, Guillermo Montalban-Bravo, Kelly A Soltysiak, Sherry Pierce, Carlos Bueso-Ramos, Jorge Cortes, Koji Sasaki |
Journal | Clinical lymphoma, myeloma & leukemia
(Clin Lymphoma Myeloma Leuk)
Vol. 21
Issue 5
Pg. 338-344
(05 2021)
ISSN: 2152-2669 [Electronic] United States |
PMID | 33597098
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Copyright | Copyright © 2021 Elsevier Inc. All rights reserved. |
Chemical References |
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Topics |
- Adult
- Core Binding Factors
(metabolism)
- Female
- Humans
- Leukemia, Myelogenous, Chronic, BCR-ABL Positive
(therapy)
- Male
- Middle Aged
- Philadelphia Chromosome
- Retrospective Studies
- Treatment Outcome
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