Synopsis

 mostly in Asia and South America, but exceptionally rare in western countries.
 polymorphic neoplastic infiltrate with an angioinvasive and angiodestructive pattern
 diffuse dense infiltrate of atypical intermediate-sized and large lymphoid cells
 scattered immunoblasts with angiocentric
 angiodestructive growth
 extensive zonal necrosis

Immunochemistry

 CD2+
 CD56+
 cytoplasmic CD3varepsilon+
 T-cell receptor gene is germline
 clonal Epstein-Barr virus (EBV) infection

Localization

 nasal NK-cell lymphoma
 non-nasal NK-cell lymphoma
 aggressive NK-cell lymphoma/leukemia

Predisposion

 AIDS
 immunosuppression for allograft (#11727268#)

• renal allograft (#11727268#)

Variants

 blastic NK-cell lymphoma (agranular CD4+ CD56+ hematodermic neoplasm) (CD3- CD4+ CD56+ CD43+ HLA-DR+ cell surface phenotype) (#12131152#)

Bioogy

 EBV DNA load is of prognostic significance and may be useful in the stratification of patients for various treatment modalities. (#16179910#)

Differential diagnosis

 angiocentric, angiodestructive CD56+ CD3+ peripheral T-cell lymphoma (#15794680#)

References

 Kwong YL. Natural killer-cell malignancies: diagnosis and treatment. Leukemia. 2005 Sep 22; PMID: #16179910#

 Shaw PH, Cohn SL, Morgan ER, Kovarik P, Haut PR, Kletzel M, Murphy SB. Natural killer cell lymphoma: report of two pediatric cases, therapeutic options, and review of the literature. Cancer. 2001 Feb 15;91(4):642-6. PMID: #11241229#