Identification of a novel SEPT9-ABL1 fusion gene in a patient with T-cell prolymphocytic leukemia

• We herein report a case of T-PLL with a novel SEPT9-ABL1 fusion.
• Ten ABL fusions, including SEPT9-ABL1, have so far been reported.
• The current case was resistant to tyrosine kinase inhibitors.

T-cell prolymphocytic leukemia (T-PLL), a rare type of peripheral T-cell leukemia, is characterized by marked splenomegaly with rapidly progressive lymphocytosis and a poor prognosis. Nine kinds of ABL1 chimeric genes have been identified in various kinds of hematological malignancies, such as chronic myeloid leukemia and B- or T-lymphoblastic leukemia. However, there have been no reports describing T-PLL cases with ABL1 rearrangements. We herein report a case of T-PLL with a novel SEPT9-ABL1 fusion gene which induced strong resistance to tyrosine kinase inhibitors such as imatinib and dasatinib.