Title

De Novo CD3 Negative Hepatosplenic T-cell Lymphoma Diagnostic Challenges and Pitfalls

Authors

Authors

L. H. Kapur; Y. Khaled; M. Solh; D. Ward;C. C. Chang

Comments

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Abbreviated Journal Title

Arch. Pathol. Lab. Med.

Keywords

OF-THE-LITERATURE; LEUKEMIA; DISEASE; Medical Laboratory Technology; Medicine, Research & Experimental; Pathology

Abstract

Hepatosplenic T-cell lymphoma is a rare and aggressive peripheral T-cell malignancy that is distinctively characterized by sinusoidal infiltration of mature medium-sized T lymphocytes in the spleen and liver. The neoplastic cells are classically surface CD3(+), CD2(+), CD5(-), CD4(-), and CD8(+/-) and manifest variable expression of markers associated with natural killer (NK) cells such as CD16 and CD56. In this article, we report the first case to date of a newly diagnosed de novo surface CD3(-) hepatosplenic T-cell lymphoma with circulating blastlike neoplastic cells expressing NK-cell-associated markers. The lack of surface CD3 expression, together with the expression of NK-cell-associated markers and the leukemic presentation, leads to significant diagnostic challenges in differentiating this CD3(-) hepatosplenic T-cell lymphoma from NK-cell neoplasms, in particular aggressive NK-cell leukemia. The related literature is reviewed, and the approaches for adequate diagnosis of this novel situation are described.

Journal Title

Archives of Pathology & Laboratory Medicine

Volume

138

Issue/Number

7

Publication Date

1-1-2014

Document Type

Article

Language

English

First Page

969

Last Page

973

WOS Identifier

WOS:000339593100022

ISSN

0003-9985

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