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Blastic natural killer cell lymphoma/leukemia (CD56-positive blastic tumor)
Prognostication and categorization according to anatomic sites of involvement
Article first published online: 5 JUL 2005
Copyright © 2005 American Cancer Society
Volume 104, Issue 5, pages 1022–1031, 1 September 2005
How to Cite
Suzuki, R., Nakamura, S., Suzumiya, J., Ichimura, K., Ichikawa, M., Ogata, K., Kura, Y., Aikawa, K., Teshima, H., Sako, M., Kojima, H., Nishio, M., Yoshino, T., Sugimori, H., Kawa, K. and Oshimi, K. (2005), Blastic natural killer cell lymphoma/leukemia (CD56-positive blastic tumor). Cancer, 104: 1022–1031. doi: 10.1002/cncr.21268
- Issue published online: 17 AUG 2005
- Article first published online: 5 JUL 2005
- Manuscript Revised: 8 APR 2005
- Manuscript Received: 19 JAN 2005
- Manuscript Accepted: 14 APR 2004
- Ministry of Health and Welfare
- Ministry of Education, Science and Culture, Japan
- natural killer cell;
- terminal deoxynucleotidyl transferase;
Blastic natural killer (NK) cell lymphoma/leukemia (BNKL) is an immature CD56-positive neoplasm, which was recognized recently and characterized by systemic proliferation of tumor cells including skin, lymph node, and bone marrow.
The current study analyzed 47 patients with BNKL (27 had leukemias and 20 had lymphomas). Patient data were collected for the survey of the NK-Cell Tumor Study Group.
There were 33 males and 14 females, with a median age of 53 years (range, 3 months to 89 years). There were few clinicopathologic differences between the leukemia and lymphoma types. Cutaneous involvement was noted at diagnosis in 28 patients, who presented a tendency for older age of onset (median: 56 vs. 46 years, P = 0.11) than patients with noncutaneous BNKL. Cutaneous BNKL showed less frequent mediastinal involvement (4% vs. 53%, P = 0.0002) and less severe thrombocytopenia (P =0 .03). Phenotypic characteristics were also different, with cutaneous BNKL favoring CD4 and HLA-DR expression, and noncutaneous BNKL favoring CD16 and CD34 expression. Both groups responded well to chemotherapy for lymphoid malignancies, but disease recurrence was frequent. The prognosis of patients with noncutaneous BNKL was significantly poorer than that of patients with cutaneous BNKL (median survival: 15 vs. 25 months, P = 0.02). Multivariate analysis confirmed that cutaneous involvement was a significant and independent prognostic factor for BNKL, as were age of onset and leukocyte count.
These findings suggested that BNKL is a heterogeneous disease and contains at least two subtypes. Although further investigations are needed to settle a marker for distinction, the presence of cutaneous involvement is a useful prognostic factor. Cancer 2005. © 2005 American Cancer Society.