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Intravascular Cytotoxic T-Cell Lymphoma in a Young Immunocompetent Woman

Intravascular lymphoma (IVL) is a rare disorder characterized by the presence of large neoplastic lymphoid cells restricted to the lumens of small vessels with a predilection for the skin and the central nervous system. While the vast majority of cases involving IVL are of B-cell lineage, the diseas...

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Autores principales: Jang, Yong Hyun, Lee, Seok-Jong, Choi, Yoon Hyuk, Lee, Weon Ju, Kim, Do Won, Kim, Jeongshik, Park, Tae In, Chae, Yee Soo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Korean Dermatological Association; The Korean Society for Investigative Dermatology 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4135106/
https://www.ncbi.nlm.nih.gov/pubmed/25143680
http://dx.doi.org/10.5021/ad.2014.26.4.496
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author Jang, Yong Hyun
Lee, Seok-Jong
Choi, Yoon Hyuk
Lee, Weon Ju
Kim, Do Won
Kim, Jeongshik
Park, Tae In
Chae, Yee Soo
author_facet Jang, Yong Hyun
Lee, Seok-Jong
Choi, Yoon Hyuk
Lee, Weon Ju
Kim, Do Won
Kim, Jeongshik
Park, Tae In
Chae, Yee Soo
author_sort Jang, Yong Hyun
collection PubMed
description Intravascular lymphoma (IVL) is a rare disorder characterized by the presence of large neoplastic lymphoid cells restricted to the lumens of small vessels with a predilection for the skin and the central nervous system. While the vast majority of cases involving IVL are of B-cell lineage, the disease rarely affects the T-cell, the histiocytes, and the natural killer cells. We report a case of intravascular T-cell lymphoma (IVTL) associated with Epstein-Barr virus (EBV). A 23-year-old healthy woman presented with tender indurated erythematous patches with overlying telangiectasia on her right breast, abdomen, both the upper and the lower extremities and the back for 3 months. The pathology revealed an infiltration of dermal and subcutaneous vessels by large and atypical lymphoid cells with immunohistochemical features of the T-cell lineage with a cytotoxic phenotype (CD3+, CD8+, granzyme B+, TIA-1+, CD4-, CD5-, CD20-, CD56-). Interestingly, the DNA extracted from the skin biopsies demonstrated evidence of a monoclonal immunoglobulin heavy chain gene rearrangement, but no T-cell receptor gene rearrangement was found. In situ hybridization study for EBV-encoded RNA was positive. She was diagnosed with an EBV-associated IVTL. The patient's skin lesions were refractory to the combination of chemotherapy and autologous stem cell transplant, and she expired. The findings in the present case may highlight the unique clinicopathologic aspects of EBV-associated cytotoxic IVTL that occurred in a young, immunocompetent woman.
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spelling pubmed-41351062014-08-20 Intravascular Cytotoxic T-Cell Lymphoma in a Young Immunocompetent Woman Jang, Yong Hyun Lee, Seok-Jong Choi, Yoon Hyuk Lee, Weon Ju Kim, Do Won Kim, Jeongshik Park, Tae In Chae, Yee Soo Ann Dermatol Case Report Intravascular lymphoma (IVL) is a rare disorder characterized by the presence of large neoplastic lymphoid cells restricted to the lumens of small vessels with a predilection for the skin and the central nervous system. While the vast majority of cases involving IVL are of B-cell lineage, the disease rarely affects the T-cell, the histiocytes, and the natural killer cells. We report a case of intravascular T-cell lymphoma (IVTL) associated with Epstein-Barr virus (EBV). A 23-year-old healthy woman presented with tender indurated erythematous patches with overlying telangiectasia on her right breast, abdomen, both the upper and the lower extremities and the back for 3 months. The pathology revealed an infiltration of dermal and subcutaneous vessels by large and atypical lymphoid cells with immunohistochemical features of the T-cell lineage with a cytotoxic phenotype (CD3+, CD8+, granzyme B+, TIA-1+, CD4-, CD5-, CD20-, CD56-). Interestingly, the DNA extracted from the skin biopsies demonstrated evidence of a monoclonal immunoglobulin heavy chain gene rearrangement, but no T-cell receptor gene rearrangement was found. In situ hybridization study for EBV-encoded RNA was positive. She was diagnosed with an EBV-associated IVTL. The patient's skin lesions were refractory to the combination of chemotherapy and autologous stem cell transplant, and she expired. The findings in the present case may highlight the unique clinicopathologic aspects of EBV-associated cytotoxic IVTL that occurred in a young, immunocompetent woman. Korean Dermatological Association; The Korean Society for Investigative Dermatology 2014-08 2014-07-31 /pmc/articles/PMC4135106/ /pubmed/25143680 http://dx.doi.org/10.5021/ad.2014.26.4.496 Text en Copyright © 2014 The Korean Dermatological Association and The Korean Society for Investigative Dermatology http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Jang, Yong Hyun
Lee, Seok-Jong
Choi, Yoon Hyuk
Lee, Weon Ju
Kim, Do Won
Kim, Jeongshik
Park, Tae In
Chae, Yee Soo
Intravascular Cytotoxic T-Cell Lymphoma in a Young Immunocompetent Woman
title Intravascular Cytotoxic T-Cell Lymphoma in a Young Immunocompetent Woman
title_full Intravascular Cytotoxic T-Cell Lymphoma in a Young Immunocompetent Woman
title_fullStr Intravascular Cytotoxic T-Cell Lymphoma in a Young Immunocompetent Woman
title_full_unstemmed Intravascular Cytotoxic T-Cell Lymphoma in a Young Immunocompetent Woman
title_short Intravascular Cytotoxic T-Cell Lymphoma in a Young Immunocompetent Woman
title_sort intravascular cytotoxic t-cell lymphoma in a young immunocompetent woman
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4135106/
https://www.ncbi.nlm.nih.gov/pubmed/25143680
http://dx.doi.org/10.5021/ad.2014.26.4.496
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