NK-cell lymphoma

Digital case

 JRC:2969 : Blastic natural killer cell malignant lymphoma

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