Chronic Lymphoproliferative Disorder of NK Cells

​

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.

​

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

​

References

​

1. Zhang R, Shah MV, Loughran TP, Jr. The root of many evils: indolent large granular lymphocyte leukaemia and associated disorders. Hematol Oncol 2010; 28: 105–117.PubMed

2. Loughran TP, Jr, Kadin ME, Starkebaum G et al. Leukemia of large granularlymphocytes: association with clonal chromosomal abnormalities and autoimmune

neutropenia, thrombocytopenia, and hemolytic anemia. Ann Intern Med 1985;102: 169–175.PubMed

3. Villamor N, Morice WG, Chan WC, Foucar K. Chronic lymphoproliferative disordersof NK cells. In Swerdlow SH et al. (eds), WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. IARD Lyon 2008; 274–275.

4. Lamy T, Loughran TP, Jr. How I treat LGL leukemia. Blood 2011; 117: 2764–2774.PubMed

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

​

​

​