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Chronic Lymphoproliferative Disorder of NK Cells
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Chronic lymphoproliferative disorder is characterized by a persistent NK-cell lymphocytosis (>2000/μL, >6 months) with an indolent clinical course. In the vast majority of patients, there are no clinical symptoms and NK-lymphocytosisPersistent large granular lymphocytosis (>2000/μL). The large granular lymphocytes show abundant cytoplasm with azurophilic granules.
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Immunophenotype:
Positive for CD16, CD56 (dim), aberrent expression of CD2, CD7, CD57 with normal NK cells, often cytoplasmic CD3ε, TIA1, granzyme B and negative for surface CD3, EBV
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References
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neutropenia, thrombocytopenia, and hemolytic anemia. Ann Intern Med 1985;102: 169–175.PubMed
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5. Gattazzo C, Teramo A, Miorin M et al. Lack of expression of inhibitory KIR3DL1receptor in patients with natural killer cell-type lymphoproliferative disease of
granular lymphocytes. Haematologica 2010; 95: 1722–1729.PubMed
6. Epling-Burnette PK, Painter JS, Chaurasia P et al. Dysregulated NK receptorexpression in patients with lymphoproliferative disease of granular lymphocytes.
Blood 2004; 103: 3431–3439.PubMed
7. Gentile TC, Uner AH, Hutchison RE et al. CD3+, CD56+ aggressive variant of large granular lymphocyte leukemia. Blood 1994; 84: 2315–2321.PubMed
8. Suzuki R, Suzumiya J, Nakamura S et al. Aggressive natural killer-cell leukemiarevisited: large granular lymphocyte leukemia of cytotoxic NK cells. Leukemia
2004; 18: 763–770.PubMed
9. Semenzato G, Marino F, Zambello R. State of the art in natural killer cellmalignancies. Int J Lab Hematol 2012; 34: 117–128.PubMed
10. Tefferi A, Li CY, Witzig TE et al. Chronic natural killer cell lymphocytosis: adescriptive clinical study. Blood 1994; 84: 2721–2725.PubMed
11. Tefferi A. Chronic natural killer cell lymphocytosis. Leuk Lymphoma 1996; 20:245–248.12. Rabbani GR, Phyliky RL, Tefferi A. A long-term study of patients with chronicnatural killer cell lymphocytosis. Br J Haematol 1999; 106: 960–966 PubMed
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