Published online Dec 6, 2021. doi: 10.12998/wjcc.v9.i34.10708
Peer-review started: June 6, 2021
First decision: June 25, 2021
Revised: July 7, 2021
Accepted: August 23, 2021
Article in press: August 23, 2021
Published online: December 6, 2021
Core Tip: Aggressive natural killer cell leukemia (ANKL) is a rare natural killer (NK) cell neoplasm characterized by systemic infiltration of Epstein–Barr virus and rapid development. Diagnosis and treatment of ANKL can be challenging due to its rapid development, rare nature, and varied clinical manifestations. Extranodal nasal NK/T cell leukemia, indolent NK cell lymphoproliferative disease, and T-large granular lymphoblastic leukemia must be considered in differential diagnosis. Standard therapy for ANKL has not been established because of its rare nature, rapid development, and poor prognosis. This is the first reported case of ANKL associated with hemophagocytic lymphohistiocytosis and rare skin lesions simultaneously.