Objective To observe the clinical features and therapeutic effect of 11p15/NUP98 rearrange-ments in acute leukemia. Methods A total of 598 newly diagnosed acute leukemia patients were detected by con-ventional cytogenetics analysis and fluorescence in situ hybridization(FISH)with the NUP98 double color probe, and the clinical data were analyzed retrospectively in the patients with 11p15/NUP98 abnormality. Results Six cases with 11p15/NUP98 rearrangement were found with a median age of 39 years old,one patient is male,the oth-ers are females. Three patients had acute monocytic leukemia(M5),one patient had acute monocytic leukemia (M2),one patient had acute monocytic leukemia(M4),and one patient had acute lymphoblastic leukemia. 11p15/NUP98 abnormality was detectable in all the patients. The median survival in all the patients was 9 months. Con-clusions Acute leukaemia with 11p15 abnormality frequently involves NUP98 gene and mainly occurrs in women. Patients with lower median age mainly developed in acute monocytic leukemia. The major clinical manifestations are anemia,low platelets and hyperleukocytosis. Acute leukemia with 11p15/NUP98 rearrangement is poorly re-sponsive to routine chemotherapies and to allogeneic hematopoietic stem cell transplantation,and thus has poor prognosis.