The pathology of NK-cell lymphomas and leukemias

Document Type

Journal Article

Publication Date

1-1-2005

Journal

Advances in Anatomic Pathology

Volume

12

Issue

1

DOI

10.1097/01.pap.0000151318.34752.80

Keywords

Cytogenetics; Dendritic cell; Epstein-Barr virus; Hemophagocytic syndrome; Leukemia; Lymphoma; Natural killer cell; T cell

Abstract

Natural killer (NK) cell lymphomas and leukemias are a rare but clinically important group of neoplasms. Most of these tumors are aggressive, with a high rate of mortality. They include extranodal NK/T-cell lymphomas of nasal type and aggressive NK-cell leukemias. Both are Epstein-Barr virus (EBV) associated and show similar epidemiologic features. A closely related entity seen mainly in children is hydroa vacciniforme-like lymphoma, which also is EBV positive. EBV influences the pathophysiology of these tumors, through the induction of cytokines and chemokines. The differential diagnosis of NK-cell malignancies includes fulminant EBV-associated T-cell lymphoproliferative disorder, a condition referred to in the past as fatal infectious mononucleosis. Benign proliferations of NK cells can be seen in association with viral infection. The disease formerly referred to as blastic NK-cell lymphoma is now considered to be a malignancy derived from a dendritic cell precursor. Copyright © 2004 by Lippincott Williams & Wilkins.

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