1. An Enteropathy-like Indolent NK-Cell Proliferation Presenting in the Female Genital Tract.
- Author
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Krishnan R, Ring K, Williams E, Portell C, Jaffe ES, and Gru AA
- Subjects
- Adult, Female, Genetic Markers, Humans, Intestinal Diseases genetics, Intestinal Diseases immunology, Intestinal Diseases surgery, Killer Cells, Natural immunology, Lymphoproliferative Disorders genetics, Lymphoproliferative Disorders immunology, Lymphoproliferative Disorders surgery, Treatment Outcome, Vagina immunology, Vagina surgery, Vaginal Diseases genetics, Vaginal Diseases immunology, Vaginal Diseases surgery, Cell Proliferation, Intestinal Diseases pathology, Killer Cells, Natural pathology, Lymphoproliferative Disorders pathology, Vagina pathology, Vaginal Diseases pathology
- Abstract
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.
- Published
- 2020
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