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Sézary Syndrome with CD4/CD8 Double-Negative Neoplastic T Cells in Peripheral Blood

Sézary syndrome is a rare leukemic type of cutaneous T-cell lymphoma characterized by the presence of neoplastic T cells with cerebriform nuclei (Sézary cells) in the skin, lymph nodes, and peripheral blood. Typical Sézary cells have a CD3(+)CD4(+)CD8(–) phenotype; however, in cases of the aberrant...

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Autores principales: Kageyama, Yuki, Tsuda, Kenshiro, Nato, Yuma, Nagaharu, Keiki, Suzuki, Kazutaka, Sawaki, Akihiko, Miyashita, Hiroyuki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8189774/
https://www.ncbi.nlm.nih.gov/pubmed/34123441
http://dx.doi.org/10.1155/2021/5527725
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author Kageyama, Yuki
Tsuda, Kenshiro
Nato, Yuma
Nagaharu, Keiki
Suzuki, Kazutaka
Sawaki, Akihiko
Miyashita, Hiroyuki
author_facet Kageyama, Yuki
Tsuda, Kenshiro
Nato, Yuma
Nagaharu, Keiki
Suzuki, Kazutaka
Sawaki, Akihiko
Miyashita, Hiroyuki
author_sort Kageyama, Yuki
collection PubMed
description Sézary syndrome is a rare leukemic type of cutaneous T-cell lymphoma characterized by the presence of neoplastic T cells with cerebriform nuclei (Sézary cells) in the skin, lymph nodes, and peripheral blood. Typical Sézary cells have a CD3(+)CD4(+)CD8(–) phenotype; however, in cases of the aberrant loss of antigens on Sézary cells, especially the loss of critically important T-cell antigens such as CD4, there is a possibility of misdiagnosing the disease or underestimating the tumor burden of the disease. Here, we report a rare case of Sézary syndrome with CD4/CD8 double-negative Sézary cells in the peripheral blood. Most of the Sézary cells in the peripheral blood had lost CD4 expression, and we diagnosed the disease and evaluated the tumor burden by multicolor flow cytometry. Intriguingly, the Sézary cells showed a typical CD4(+)CD8(–)CD7(–) phenotype in the skin even though the cells in the peripheral blood lacked CD4. The patient responded well to treatment with bexarotene and narrow-band ultraviolet B therapy. Analysis by multicolor flow cytometry is essential to diagnose this rare type of Sézary syndrome and evaluate the tumor burden.
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spelling pubmed-81897742021-06-11 Sézary Syndrome with CD4/CD8 Double-Negative Neoplastic T Cells in Peripheral Blood Kageyama, Yuki Tsuda, Kenshiro Nato, Yuma Nagaharu, Keiki Suzuki, Kazutaka Sawaki, Akihiko Miyashita, Hiroyuki Case Rep Hematol Case Report Sézary syndrome is a rare leukemic type of cutaneous T-cell lymphoma characterized by the presence of neoplastic T cells with cerebriform nuclei (Sézary cells) in the skin, lymph nodes, and peripheral blood. Typical Sézary cells have a CD3(+)CD4(+)CD8(–) phenotype; however, in cases of the aberrant loss of antigens on Sézary cells, especially the loss of critically important T-cell antigens such as CD4, there is a possibility of misdiagnosing the disease or underestimating the tumor burden of the disease. Here, we report a rare case of Sézary syndrome with CD4/CD8 double-negative Sézary cells in the peripheral blood. Most of the Sézary cells in the peripheral blood had lost CD4 expression, and we diagnosed the disease and evaluated the tumor burden by multicolor flow cytometry. Intriguingly, the Sézary cells showed a typical CD4(+)CD8(–)CD7(–) phenotype in the skin even though the cells in the peripheral blood lacked CD4. The patient responded well to treatment with bexarotene and narrow-band ultraviolet B therapy. Analysis by multicolor flow cytometry is essential to diagnose this rare type of Sézary syndrome and evaluate the tumor burden. Hindawi 2021-06-01 /pmc/articles/PMC8189774/ /pubmed/34123441 http://dx.doi.org/10.1155/2021/5527725 Text en Copyright © 2021 Yuki Kageyama et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Kageyama, Yuki
Tsuda, Kenshiro
Nato, Yuma
Nagaharu, Keiki
Suzuki, Kazutaka
Sawaki, Akihiko
Miyashita, Hiroyuki
Sézary Syndrome with CD4/CD8 Double-Negative Neoplastic T Cells in Peripheral Blood
title Sézary Syndrome with CD4/CD8 Double-Negative Neoplastic T Cells in Peripheral Blood
title_full Sézary Syndrome with CD4/CD8 Double-Negative Neoplastic T Cells in Peripheral Blood
title_fullStr Sézary Syndrome with CD4/CD8 Double-Negative Neoplastic T Cells in Peripheral Blood
title_full_unstemmed Sézary Syndrome with CD4/CD8 Double-Negative Neoplastic T Cells in Peripheral Blood
title_short Sézary Syndrome with CD4/CD8 Double-Negative Neoplastic T Cells in Peripheral Blood
title_sort sézary syndrome with cd4/cd8 double-negative neoplastic t cells in peripheral blood
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8189774/
https://www.ncbi.nlm.nih.gov/pubmed/34123441
http://dx.doi.org/10.1155/2021/5527725
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