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Abnormal immunophenotype provides a key diagnostic marker: a report of 29 cases of de novo aggressive natural killer cell leukemia

  • Chunrui Li
    Affiliations
    Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China
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  • Ye Tian
    Affiliations
    Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China
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  • Jue Wang
    Affiliations
    Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China
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  • Li Zhu
    Affiliations
    Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China
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  • Liang Huang
    Affiliations
    Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China
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  • Na Wang
    Affiliations
    Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China
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  • Danmei Xu
    Affiliations
    Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China
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  • Yang Cao
    Affiliations
    Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China
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  • Jianyong Li
    Correspondence
    Reprint requests: Jianfeng Zhou, PhD, MD, Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1095 Jie-Fang Avenue, Wuhan, Hubei, 430030, PR China, and Jianyong Li, PhD, MD, Department of Haematology, the First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, P.R. China
    Affiliations
    Department of Haematology, the First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, PR China
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  • Jianfeng Zhou
    Correspondence
    Reprint requests: Jianfeng Zhou, PhD, MD, Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1095 Jie-Fang Avenue, Wuhan, Hubei, 430030, PR China, and Jianyong Li, PhD, MD, Department of Haematology, the First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, P.R. China
    Affiliations
    Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, PR China
    Search for articles by this author
Published:January 24, 2014DOI:https://doi.org/10.1016/j.trsl.2014.01.010
      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.

      Abbreviations:

      ANKL (aggressive natural killer cell leukemia), AspaMetDex (L-asparaginase, high-dose of methotrexate and dexamethasone), BM (bone marrow), CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), CLPD-NK (chronic lymphoproliferative disorders of natural killer cells), CT (computed tomography), EBV (Epstein-Barr virus), EBER (Epstein-Barr virus-encoded RNA), ENKTL (extranodal natural killer/T-cell lymphoma, nasal type), FDG (fluorodeoxyglucose), IPI (International Prognostic Index), KIR (killer cell immunoglobulin receptor), LDH (lactate dehydrogenase), LGL (large granular lymphocyte), LMP1 (Latent membrane protein 1), NK (natural killer), PB (peripheral blood), PET (positron emission tomography), PCR (polymerase chain reaction), SSC (linear side scatter), TCR (T-cell receptor)
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