Although the presence of ETNK1 mutations has been considered as a relatively specific finding for aCML, leading to their inclusion in the 2016 WHO classification as a support criterion for the diagnosis of the disease, they have also been found in chronic myelomonocytic leukemia (CMML) and systemic mastocytosis with eosinophilia (34) while they were recently described in diffuse large B-cell lymphoma (35). Here, ETNK1 is linked to atypical chronic myeloid leukemia, BCR-ABL1 negative.