title: Abnormal immunophenotype provides a key diagnostic marker: a report of 29 cases of de novo aggressive natural killer cell leukemia.
abstract: Aggressive natural killer (NK) cell leukemia (ANKL) is a systemic neoplastic proliferation of NK cells with an aggressive clinical course. Currently, the diagnosis of ANKL remains challenging. In the current study, we report the clinical, laboratory, immunophenotypic, and genetic findings from 29 cases of de novo ANKL in a single center and evaluate the relative contribution of these features to the diagnosis of ANKL. Clinical features, laboratory findings, morphologic, cytogenetic features, and Epstein-Barr virus status were important factors for diagnosing aggressive NK cell leukemia. On the other hand, ANKL displays a strikingly abnormal immunophenotype in contrast to nonneoplastic NK cells. The immunophenotype of ANKL cells may differ from reactive NK cells in 4 respects. First, the CD45/linear side scatter gating of flow cytometry allows the initial identification of neoplastic subpopulations for additional immunophenotypic analysis in half of ANKL cases. Second, unusual expression of surface antigens in ANKL cells was a prominent feature. Third, the clonality of ANKL cells could be identified using antibodies against CD158a/h, CD158b, or CD158e. Last, the positive rate of Ki-67 expression in ANKL cells was generally high. Based on these findings, we provide an objective marker based on clinical data for the definite diagnosis of ANKL.
authors: Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China.
authors: Chunrui
authors: C
authors: Li
authors: Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China.
authors: Ye
authors: Y
authors: Tian
authors: Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China.
authors: Jue
authors: J
authors: Wang
authors: Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China.
authors: Li
authors: L
authors: Zhu
authors: Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China.
authors: Liang
authors: L
authors: Huang
authors: Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China.
authors: Na
authors: N
authors: Wang
authors: Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China.
authors: Danmei
authors: D
authors: Xu
authors: Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China.
authors: Yang
authors: Y
authors: Cao
authors: Department of Haematology, the First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, PR China. Electronic address: lijianyonglm@medmail.com.cn.
authors: Jianyong
authors: J
authors: Li
authors: Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China. Electronic address: jfzhou@tjh.tjmu.edu.cn.
authors: Jianfeng
authors: J
authors: Zhou
mesh_terms: D000293:Adolescent; D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D016130:Immunophenotyping; D019394:Ki-67 Antigen / Q000378:metabolism; D007694:Killer Cells, Natural / Q000276:immunology*; D007945:Leukemia, Lymphoid / Q000175:diagnosis / Q000235:genetics / Q000276:immunology*; D017493:Leukocyte Common Antigens / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D049268:Positron-Emission Tomography; D054342:Receptors, KIR2DL1 / Q000378:metabolism; D054344:Receptors, KIR2DL3 / Q000378:metabolism; D054341:Receptors, KIR3DL1 / Q000378:metabolism; D014057:Tomography, X-Ray Computed; D057170:Translational Research, Biomedical; D014763:Viral Matrix Proteins / Q000378:metabolism; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; C037662:EBV-associated membrane antigen, Epstein-Barr virus; D019394:Ki-67 Antigen; D054342:Receptors, KIR2DL1; D054344:Receptors, KIR2DL3; D054341:Receptors, KIR3DL1; D014763:Viral Matrix Proteins; D017493:Leukocyte Common Antigens; C516769:PTPRC protein, human