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The t(8;21)(q22;q22) is one of the most common chromosomal abnormalities in acute myeloid leukemia (AML). Approximately 3–4% of AML cases are associated with additional chromosomal abnormalities. Their impact on the prognosis of the disease remains to be established. Here we report a case of t(8;10;21) AML with mutated c-KIT that shared key morphological features with classical t(8;21) leukemias, including the M2 morphology pattern and CD34, HLA-DR phenotype. The 63-year-old female was treated with two inductioncontaining Daunoribicine and Cytarabine and four cycles of intermediate-dose Cytarabine (1.5 g/m2) and achieved long-lasting remission.