Guillermo Montalban-Bravo, Rashmi Kanagal-Shamanna, Koji Sasaki, Lucia Masarova, Kiran Naqvi, Elias Jabbour, Courtney D DiNardo, Koichi Takahashi, Marina Konopleva, Naveen Pemmaraju, Tapan M Kadia, Farhad Ravandi, Naval Daver, Gautam Borthakur, Zeev Estrov, Joseph D Khoury, Sanam Loghavi, Kelly A Soltysiak, Sherry Pierce, Carlos Bueso-Ramos, Keyur P Patel, Srdan Verstovsek, Hagop M Kantarjian, Prithviraj Bose, Guillermo Garcia-Manero
BACKGROUND: There are limited data on the clonal mechanisms underlying leukemogenesis, prognostic factors, and optimal therapy for atypical chronic myeloid leukemia (aCML). METHODS: The authors evaluated the clinicopathologic features, outcomes, and responses to therapy of 65 patients with aCML. The median age was 67 years (range, 46-89 years). RESULTS: The most frequently mutated genes included ASXL1 (83%), SRSF2 (68%), and SETBP1 (58%). Mutations in SETBP1, SRSF2, TET2, and GATA2 appeared at variant allele frequencies (VAFs) greater than 40%, whereas other RAS pathway mutations were more likely to appear at low VAFs...
September 1, 2021: Cancer