Open AccessAn Enteropathy-like Indolent NK-Cell Proliferation Presenting in the Female Genital Tract
Author(s) -
Krishnan Rahul,
Kari L. Ring,
Eli S. Williams,
Craig A. Portell,
Elaine S. Jaffe,
Alejandro A. Gru
Publication year - 2019
Publication title -
the american journal of surgical pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.625
H-Index - 210
eISSN - 1532-0979
pISSN - 0147-5185
DOI - 10.1097/pas.0000000000001387
Subject(s) - enteropathy , lymphoma , lymphoproliferative disorders , pathology , genitourinary system , immunology , medicine , gastrointestinal tract , natural killer cell , population , immunophenotyping , biology , disease , cytotoxic t cell , flow cytometry , biochemistry , environmental health , in vitro
Natural killer (NK) cell enteropathy is a lymphoproliferative disorder, initially described by Mansoor and colleagues, that presents in the gastrointestinal tract, and is often mistaken for extranodal NK/T-cell lymphoma on first assessment. This population of cells in this process have an NK-cell phenotype (CD3, CD56, CD2, CD7), lacks evidence of Epstein-Barr virus infection, has germline rearrangement of the T-cell receptor, and a very indolent clinical course. Indeed, many of such patients had been originally diagnosed as having an NK/T-cell lymphoma, and subsequently received chemotherapy. We report a unique case where an indolent lymphoproliferative disorder with features that resemble NK-cell enteropathy is encountered for the first time outside the gastrointestinal tract, specifically in the female genitourinary tract. We provide morphologic, immunophenotypic, and molecular documentation of such, in association with a completely indolent clinical behavior of this type of process.