FGFR1 rearrangements, including FGFR1::ZMYM2 due to t(8;13)(p11;q12), are associated with aggressive clinical phenotypes with, in the absence of treatment, constant and rapid progression (in one to two years) towards a high-grade hematological malignancy (acute myeloid leukemia, lymphoblastic lymphoma) resistant to standard TKIs (including ponatinib). Here, FGFR1 is linked to lymphoblastic lymphoma.