An 81-year-old female was referred…A diagnosis of myelodysplastic syndrome with multilineage dysplasia (MDS-MLD) was made...Treatment with low doses of the tyrosine kinase inhibitor (TKI), imatinib mesylate (100 mg), was initiated...Our results suggest that complete hematologic recovery in response to imatinib mesylate suggests a critical role for the BCR-ABL1 fusion in the pathogenesis of this disease.