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International Journal of Hematology

, Volume 85, Issue 1, pp 18–25 | Cite as

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

  • John Ryder
  • Xiaoqin Wang
  • Liming Bao
  • Sherilyn A. Gross
  • Fu Hua
  • Richard D. Irons
Article

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.

Key words

Natural killer cell Leukemia CD56 EBV 

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Copyright information

© The Japanese Society of Hematology 2007

Authors and Affiliations

  • John Ryder
    • 1
    • 2
    • 6
  • Xiaoqin Wang
    • 1
    • 3
  • Liming Bao
    • 1
    • 8
  • Sherilyn A. Gross
    • 1
    • 7
  • Fu Hua
    • 4
  • Richard D. Irons
    • 1
    • 2
    • 5
    • 6
    • 7
  1. 1.Sino-US Joint Clinical and Molecular LaboratoryChina
  2. 2.Department of PathologyChina
  3. 3.Huashan HospitalChina
  4. 4.School of Public HealthChina
  5. 5.Institutes of Biomedical SciencesFudan UniversityShanghaiChina
  6. 6.Department of Pathology, School of MedicineChina
  7. 7.Department of Pharmaceutical Sciences, School of PharmacyUniversity of Colorado at Denver and Health Sciences CenterDenverUSA
  8. 8.Division of Human GeneticsCincinnati Children’s Hospital Medical Center and University of Cincinnati College of MedicineCincinnatiUSA

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