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
Aggressive natural killer cell leukemia (ANKL) is a rare natural killer cell neoplasm characterized by systemic infiltration of Epstein–Barr virus and rapidly progressive clinical course. ANKL can be accompanied with hemophagocytic lymphohistiocytosis (HLH). Here, we report a case of ANKL with rare skin lesions as an earlier manifestation, accompanied with HLH, and review the literature in terms of etiology, clinical manifestation, diagnosis and treatment.
A 30-year-old woman from Northwest China presented with the clinical characteristics of jaundice, fever, erythema, splenomegaly, progressive hemocytopenia, liver failure, quantities of abnormal cells in bone marrow, and associated HLH. The immunophenotypes of abnormal cells were positive for CD2, cCD3, CD7, CD56, CD38 and negative for sCD3, CD8 and CD117. The diagnosis of ANKL complicated with HLH was confirmed. Following the initial diagnosis and supplementary treatment, the patient received chemotherapy with VDLP regimen (vincristine, daunorubicin, L-asparaginase and prednisone). However, the patient had severe adverse reactions and complication such as severe hematochezia, neutropenia, and multiple organ dysfunction syndrome, and died a few days later.
This is the first reported case of ANKL with rare skin lesions as an earlier manifestation and associated with HLH.
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.