Atypical chronic myeloid leukemia (aCML) is a BCR-ABL1 negative myelodysplastic/myeloproliferative (MDS/MPN) neoplasm. Patients typically present with elevated neutrophil counts and hypercellular bone marrow, but there are no specific genetic or molecular markers available to diagnose aCML and it is therefore a diagnosis of exclusion. Atypical CML is rare and carries a poor prognosis, and there is currently no standard of care for treatment. In the absence of an available clinical trial, current consensus is for patients with a suitable donor to undergo allogeneic stem cell transplantation, and a comprehensive evaluation for driver mutations should be performed to screen for the potential use of targeted agents. Without an actionable driver mutation, hypomethylating agents are an emerging treatment option based on four reports showing complete hematologic remission in 7 of 8 patients treated with decitabine. Keywords: Atypical chronic myeloid leukemia, atypical CML, mds/mpn
Corresponding Author: James Mackey