Aggressive NK-cell leukemia

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Aggressive NK-cell leukemia is a disease with an aggressive, systemic proliferation of natural killer cells (NK cells) and a rapidly declining clinical course.

It is also called aggressive NK-cell lymphoma.  ANKL is an Epstein-Barr virus (EBV)-associated tumor most prevalent among Asian young adults. There is no standard treatment currently available for patients.

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Immunophenotyping:

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Positive for CD2, CD3, CD56, perforin, granzyme B, TIA-1, CCR5 and Negative for CD57

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                                                                                                                               Case-1

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       Immunophenotyping shows normal lymphocytes(green) and blasts cell(red). blast are positive for CD7, CD56, CD3dim/-, CD2dim/- and negative for cCD3, HLA-DR, CD5, MPO, CD79a, CD19, CD117, CD65, CD1a, CD4, CD8, CD33, CD34 and CD13

 

Reference:

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• Dearden CE, Johnson R, Pettengell R, et al. British Committee for Standards in Haematology Guidelines for the management of ma ture T-cell and NK-cell neoplasms (excluding cutaneous T-cell lymphoma) Br J Haematol. 2011;153(4):451–485. [PubMed]

• Suzuki R, Suzumiya S, Nakamura S, et al. NK-cell Tumor Study Group Aggressive natural killer-cell leukemia revisited: large granular lymphocyte leukemia of cytotoxic NK cells. Leukemia. 2004;18(4):763–770. [PubMed]

• Lamy T, Loughran TP., Jr Clinical features of large granular lymphocyte leukemia. Semin Hematol. 2003;40(3):185–195. [PubMed]

• Li C, Tian Y, Wang J, et al. Abnormal immunophenotype provides a key diagnostic marker: a report of 29 cases of de novo aggressive natural killer cell leukemia. Transl Res. 2014;163(6):565–577. [PubMed]

• Lima M, Spínola A, Fonseca S, et al. Aggressive mature natural killer cell neoplasms: report on a series of 12 European patients with emphasis on flow cytometry based immunophenotype and DNA content of neoplastic natural killer cells. Leuk Lymphoma. 2014;56(1):103–112. [PubMed]

• Ruskova A, Thula R, Chan G. Aggressive natural killer-cell leukemia: report of five cases and review of the literature. Leuk Lymphoma. 2004;45(12):2427–2438. [PubMed]

• Okamura T, Kishimoto T, Inoue M, et al. Unrelated bone marrow transplantation for Epstein-Barr virus-associated T/NK-cell lymphoproliferative disease. Bone Marrow Transplant. 2003;31(2):105–111. [PubMed]

• Jaccard A, Petit B, Girault S. L-Asparaginase-based treatment of 15 western patients with extranodal NK/T-cell lymphoma and leukemia and a review of the literature. Ann Oncol. 2009;20(1):110–116. [PubMed]

• Yamaguchi M, Suzuki R, Kwong YL, et al. Phase I study of dexamethasone, methotrexate, ifosfamide, l-asparaginase, and etoposide (SMILE) chemotherapy for advanced-stage, relapsed or refractory extranodal natural killer (NK)/T-cell lymphoma and leukemia. Cancer Sci. 2008;99(5):1016–1020. [PubMed]