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Aggressive Natural Killer Cell Leukemia: Report of a Chinese Series and Review of the Literature

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Abstract

Aggressive natural killer cell leukemia (ANKL) is a rare Epstein-Barr virus (EBV)-associated fulminating disease that is widely disseminated at diagnosis. Because of its typically extranodal presentation, differing degrees of NK cell involvement, and varying bone marrow pathology, ANKL can be confused with a reactive process. These features, coupled with a rapidly fatal course, have hampered systematic study of the pathogenesis of ANKL. Nine cases of ANKL were diagnosed and characterized by a single laboratory over a 2-year period. Constant features at presentation included disseminated disease, high fever, bone marrow involvement, and a high lactate dehydrogenase index. All cases were positive for EBV early region protein and negative for latent membrane protein 1, and all had a germline T-cell receptor gene configuration. Peripheral blood counts were variable, with severe thrombocytopenia being the most frequently encountered abnormality (7 of 9 cases). Hematophagocytosis, dyserythropoiesis, and stromal degeneration were the most frequent findings in the bone marrow. Neo-plastic cells in the bone marrow were consistently CD2+, CD56+, CD45+, CD34-, CD117-, CD4-, and surface CD3-. Most cases were HLA-DR+ (8/9) and CD8- (8/9). Complex clonal cytogenetic abnormalities were found in 8 of 9 cases. Because of its aggressive course, rapid and accurate diagnosis of ANKL is essential for a better understanding of the etiology, pathogenesis, and treatment of the disease.

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Author information

Authors and Affiliations

  1. Sino-US Joint Clinical and Molecular Laboratory, China

    John Ryder, Xiaoqin Wang, Liming Bao, Sherilyn A. Gross & Richard D. Irons

  2. Department of Pathology, China

    John Ryder & Richard D. Irons

  3. Huashan Hospital, China

    Xiaoqin Wang

  4. School of Public Health, China

    Fu Hua

  5. Institutes of Biomedical Sciences, Fudan University, Shanghai, China

    Richard D. Irons

  6. Department of Pathology, School of Medicine, China

    John Ryder & Richard D. Irons

  7. Department of Pharmaceutical Sciences, School of Pharmacy, University of Colorado at Denver and Health Sciences Center, 4200 E 9th Ave/Box C238, 80262, Denver, CO, USA

    Sherilyn A. Gross & Richard D. Irons

  8. Division of Human Genetics, Cincinnati Children’s Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati, Ohio, USA

    Liming Bao

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  1. John Ryder
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  2. Xiaoqin Wang
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Correspondence to Richard D. Irons.

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Ryder, J., Wang, X., Bao, L. et al. Aggressive Natural Killer Cell Leukemia: Report of a Chinese Series and Review of the Literature. Int J Hematol 85, 18–25 (2007). https://doi.org/10.1532/IJH97.A10612

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  • DOI: https://doi.org/10.1532/IJH97.A10612

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