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T-lymphoblastic lymphoma/leukemia without clonal TCR gene rearrangements: case report and literature review
T-lymphoblastic lymphoma (T-LBL) is a highly aggressive malignancy originating from T-lymphocyte precursors. Incidence is highest in children and adolescents. T-cell receptor (TCR) gene rearrangement is usually present. TCR gene rearrangement-negative cases are considered rare. Here, we investigated...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
AME Publishing Company
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8799306/ https://www.ncbi.nlm.nih.gov/pubmed/35116486 http://dx.doi.org/10.21037/tcr-20-2902 |
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author | Wang, Jiuju Jiang, Shanshan Zhao, Qiaojiajie Wei, Xucang Li, Wensheng |
author_facet | Wang, Jiuju Jiang, Shanshan Zhao, Qiaojiajie Wei, Xucang Li, Wensheng |
author_sort | Wang, Jiuju |
collection | PubMed |
description | T-lymphoblastic lymphoma (T-LBL) is a highly aggressive malignancy originating from T-lymphocyte precursors. Incidence is highest in children and adolescents. T-cell receptor (TCR) gene rearrangement is usually present. TCR gene rearrangement-negative cases are considered rare. Here, we investigated the clinicopathological features, differential diagnosis, therapy, and prognosis of TCR gene rearrangement-negative T-lymphoblastic lymphoma/leukemia (T-LBL/ALL) by case report and literature review. An 18-year-old male with polyglandular lymphadenopathy underwent an excisional lymph node biopsy and bone marrow aspiration that disclosed diffuse distribution of round, small to medium sized cells with scant cytoplasm, delicate chromatin, and frequent mitotic figures. Immunophenotyping showed expression of TDT, CD3, CD7, and CD5, no CD34, CD20, CD56, bcl-6, CD4, CD8, or MPO in lymph node tissue. Immunohistochemical staining for pathological consultation was performed by Streptavidin peroxidase (SP) method, EB virus coded small RNA (EBER) tested by in situ hybridization (ISH), (EBER-ISH). And flow cytometry of bone marrow aspirate showed that tumor cells expressed CD3, CD5, CD7; partial expression of CD2, CD10, CD38, TDT; and no expression of CD1a, CD34, CD4, CD8, mCD3, CD33, CD56, CD19, CD79a, HLA-DR and MPO. These findings led to the diagnosis of T-LBL/-ALL. Molecular genetic testing showed no TCR gene rearrangement. The patient received chemotherapy consisting of vinorelbine, pirarubicin, cyclophosphamide, asparaginase, and prednisone. Prophylactic chemotherapy of the central nervous system and radiotherapy of the mediastinum were also given. And responded to combined chemotherapy and radiotherapy. Although T-LBL/ALL typically features TCR gene rearrangement, rare cases without rearrangement may occur. Diagnosis is based on clinical characteristics, histopathology, immunotyping, and molecular genetics. |
format | Online Article Text |
id | pubmed-8799306 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | AME Publishing Company |
record_format | MEDLINE/PubMed |
spelling | pubmed-87993062022-02-02 T-lymphoblastic lymphoma/leukemia without clonal TCR gene rearrangements: case report and literature review Wang, Jiuju Jiang, Shanshan Zhao, Qiaojiajie Wei, Xucang Li, Wensheng Transl Cancer Res Case Report T-lymphoblastic lymphoma (T-LBL) is a highly aggressive malignancy originating from T-lymphocyte precursors. Incidence is highest in children and adolescents. T-cell receptor (TCR) gene rearrangement is usually present. TCR gene rearrangement-negative cases are considered rare. Here, we investigated the clinicopathological features, differential diagnosis, therapy, and prognosis of TCR gene rearrangement-negative T-lymphoblastic lymphoma/leukemia (T-LBL/ALL) by case report and literature review. An 18-year-old male with polyglandular lymphadenopathy underwent an excisional lymph node biopsy and bone marrow aspiration that disclosed diffuse distribution of round, small to medium sized cells with scant cytoplasm, delicate chromatin, and frequent mitotic figures. Immunophenotyping showed expression of TDT, CD3, CD7, and CD5, no CD34, CD20, CD56, bcl-6, CD4, CD8, or MPO in lymph node tissue. Immunohistochemical staining for pathological consultation was performed by Streptavidin peroxidase (SP) method, EB virus coded small RNA (EBER) tested by in situ hybridization (ISH), (EBER-ISH). And flow cytometry of bone marrow aspirate showed that tumor cells expressed CD3, CD5, CD7; partial expression of CD2, CD10, CD38, TDT; and no expression of CD1a, CD34, CD4, CD8, mCD3, CD33, CD56, CD19, CD79a, HLA-DR and MPO. These findings led to the diagnosis of T-LBL/-ALL. Molecular genetic testing showed no TCR gene rearrangement. The patient received chemotherapy consisting of vinorelbine, pirarubicin, cyclophosphamide, asparaginase, and prednisone. Prophylactic chemotherapy of the central nervous system and radiotherapy of the mediastinum were also given. And responded to combined chemotherapy and radiotherapy. Although T-LBL/ALL typically features TCR gene rearrangement, rare cases without rearrangement may occur. Diagnosis is based on clinical characteristics, histopathology, immunotyping, and molecular genetics. AME Publishing Company 2021-03 /pmc/articles/PMC8799306/ /pubmed/35116486 http://dx.doi.org/10.21037/tcr-20-2902 Text en 2021 Translational Cancer Research. All rights reserved. https://creativecommons.org/licenses/by-nc-nd/4.0/Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/. |
spellingShingle | Case Report Wang, Jiuju Jiang, Shanshan Zhao, Qiaojiajie Wei, Xucang Li, Wensheng T-lymphoblastic lymphoma/leukemia without clonal TCR gene rearrangements: case report and literature review |
title | T-lymphoblastic lymphoma/leukemia without clonal TCR gene rearrangements: case report and literature review |
title_full | T-lymphoblastic lymphoma/leukemia without clonal TCR gene rearrangements: case report and literature review |
title_fullStr | T-lymphoblastic lymphoma/leukemia without clonal TCR gene rearrangements: case report and literature review |
title_full_unstemmed | T-lymphoblastic lymphoma/leukemia without clonal TCR gene rearrangements: case report and literature review |
title_short | T-lymphoblastic lymphoma/leukemia without clonal TCR gene rearrangements: case report and literature review |
title_sort | t-lymphoblastic lymphoma/leukemia without clonal tcr gene rearrangements: case report and literature review |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8799306/ https://www.ncbi.nlm.nih.gov/pubmed/35116486 http://dx.doi.org/10.21037/tcr-20-2902 |
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