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Aggressive natural killer-cell leukemia revisited: large granular lymphocyte leukemia of cytotoxic NK cells

Leukemia volume 18, pages 763–770 (2004)Cite this article

Abstract

Aggressive natural killer-cell leukemia (ANKL) is a rare form of large granular lymphocyte leukemia, which is characterized by a systemic proliferation of NK cells. The clinical features of 22 ANKL cases were analyzed. Hepatomegaly (64%), splenomegaly (55%) and lymphadenopathy (41%) were also frequently observed. Leukemic cells were identified as CD1−, CD2+, surface CD3−, CD4−, CD5−, CD7+, CD8+/−, CD10−, CD11b+/−, CD13−, CD16+, CD19−, CD20−, CD25−, CD33−, CD34−, CD38+, CD56+, CD122+, HLA-DR+ and TCR−. Two of the 16 cases examined for CD57 were positive and three of the seven cases examined for cytoplasmic CD3. Epstein–Barr virus was detected in the tumor cells of 11 of the 13 cases examined. No common cytogenetic abnormalities were identified and 6q anomaly was detected in only one. Three of 13 patients treated with chemotherapy containing anthracycline/anthraquinone attained complete remission, in contrast to none of the eight who were treated with regimens without anthracycline. Although the overall prognosis was poor with a median survival of 58 days, those who attained remission showed better prognosis (P=0.005). These findings suggest that ANKL is an entity of mature cytotoxic NK-cell neoplasms with distinct phenotype and disease presentations. Intensive treatment for ANKL may result in a better prognosis.

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Acknowledgements

This work was supported in part by a grant-in-aid for the second-term comprehensive 10-year strategy for cancer control from the Ministry of Health and Welfare, a grant-in-aid for science on primary areas (Cancer Research), from the Ministry of Education, Science and Culture, Japan. We thank the collaborators from the following institutions for providing the patients' data and specimens: Saiseikai Maebashi Hospital; Mie University; Japanese Red Cross Nagoya First Hospital; Kitano Hospital; Dokkyo University School of Medicine; Fujita Health University School of Medicine; Hiroshima University School of Medicine; Kansai Medical University; Toride Kyodo Hospital; Tokyo Medical and Dental University; Kyushu University and a grant-in-aid for Encouragement of Young Scientists from the Ministry of Education, Science and Culture, Japan.

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Authors and Affiliations

  1. Division of Molecular Medicine, Aichi Cancer Center, Nagoya, Japan

    R Suzuki

  2. First Department of Internal Medicine, Fukuoka University School of Medicine, Fukuoka, Japan

    J Suzumiya

  3. Department of Pathology and Molecular Diagnostics, Aichi Cancer Center, Nagoya, Japan

    S Nakamura

  4. First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan

    S Aoki

  5. Department of Clinical Immunology and Hematology, Sapporo City General Hospital, Sapporo, Japan

    A Notoya

  6. First Department of Internal Medicine, The University of Tokushima, Japan

    S Ozaki

  7. Department of Hematology, Hamanomachi Hospital, Fukuoka, Japan

    H Gondo

  8. Department of Internal Medicine, Kure Kyosai Hospital, Kure, Japan

    N Hino

  9. Division of Hematology, Showa University Fujigaoka Hospital, Yokohama, Japan

    H Mori

  10. Department of Preventive Medicine, St Marianna University School of Medicine, Kawasaki, Japan

    H Sugimori

  11. Department of Pediatrics, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan

    K Kawa

  12. Department of Hematology, Juntendo University, Tokyo, Japan

    K Oshimi

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Suzuki, R., Suzumiya, J., Nakamura, S. et al. Aggressive natural killer-cell leukemia revisited: large granular lymphocyte leukemia of cytotoxic NK cells. Leukemia 18, 763–770 (2004). https://doi.org/10.1038/sj.leu.2403262

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