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 loss o...
Saved in:
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2021-01-01
|
| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2021/5527725 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832546086326108160 |
|---|---|
| author | Yuki Kageyama Kenshiro Tsuda Yuma Nato Keiki Nagaharu Kazutaka Suzuki Akihiko Sawaki Hiroyuki Miyashita |
| author_facet | Yuki Kageyama Kenshiro Tsuda Yuma Nato Keiki Nagaharu Kazutaka Suzuki Akihiko Sawaki Hiroyuki Miyashita |
| author_sort | Yuki Kageyama |
| collection | DOAJ |
| 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. |
| format | Article |
| id | doaj-art-c3dae780e75c4b1eb909165741a1f287 |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-c3dae780e75c4b1eb909165741a1f2872025-02-03T07:24:02ZengWileyCase Reports in Hematology2090-65602090-65792021-01-01202110.1155/2021/55277255527725Sézary Syndrome with CD4/CD8 Double-Negative Neoplastic T Cells in Peripheral BloodYuki Kageyama0Kenshiro Tsuda1Yuma Nato2Keiki Nagaharu3Kazutaka Suzuki4Akihiko Sawaki5Hiroyuki Miyashita6Department of Hematology, Yokkaichi Municipal Hospital, 2-2-37 Shibata, Yokkaichi 510-8567, JapanDepartment of Dermatology, Yokkaichi Municipal Hospital, 2-2-37 Shibata, Yokkaichi 510-8567, JapanDepartment of Hematology, Yokkaichi Municipal Hospital, 2-2-37 Shibata, Yokkaichi 510-8567, JapanDepartment of Hematology and Oncology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu 514-8507, JapanDepartment of Hematology, Yokkaichi Municipal Hospital, 2-2-37 Shibata, Yokkaichi 510-8567, JapanDepartment of Hematology, Yokkaichi Municipal Hospital, 2-2-37 Shibata, Yokkaichi 510-8567, JapanDepartment of Hematology, Yokkaichi Municipal Hospital, 2-2-37 Shibata, Yokkaichi 510-8567, JapanSézary 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.http://dx.doi.org/10.1155/2021/5527725 |
| spellingShingle | Yuki Kageyama Kenshiro Tsuda Yuma Nato Keiki Nagaharu Kazutaka Suzuki Akihiko Sawaki Hiroyuki Miyashita Sézary Syndrome with CD4/CD8 Double-Negative Neoplastic T Cells in Peripheral Blood Case Reports in Hematology |
| 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 | sezary syndrome with cd4 cd8 double negative neoplastic t cells in peripheral blood |
| url | http://dx.doi.org/10.1155/2021/5527725 |
| work_keys_str_mv | AT yukikageyama sezarysyndromewithcd4cd8doublenegativeneoplastictcellsinperipheralblood AT kenshirotsuda sezarysyndromewithcd4cd8doublenegativeneoplastictcellsinperipheralblood AT yumanato sezarysyndromewithcd4cd8doublenegativeneoplastictcellsinperipheralblood AT keikinagaharu sezarysyndromewithcd4cd8doublenegativeneoplastictcellsinperipheralblood AT kazutakasuzuki sezarysyndromewithcd4cd8doublenegativeneoplastictcellsinperipheralblood AT akihikosawaki sezarysyndromewithcd4cd8doublenegativeneoplastictcellsinperipheralblood AT hiroyukimiyashita sezarysyndromewithcd4cd8doublenegativeneoplastictcellsinperipheralblood |