Clinical features, laboratory characteristics, and outcome of ETP and TCRA/D aberrations in pediatric patients with T-acute lymphoblastic leukemia
Abstract Background T-cell acute lymphoblastic leukemia (T-ALL) is an aggressive malignancy with few accepted prognostic factors that limit the efficiency of therapy. The aim of the current study was to assess the clinical and laboratory features of T-cell receptor (TCR) aberrations and early T-cell...
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| Format: | Article |
| Language: | English |
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SpringerOpen
2023-06-01
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| Series: | Journal of the Egyptian National Cancer Institute |
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| Online Access: | https://doi.org/10.1186/s43046-023-00176-1 |
| _version_ | 1797801318362906624 |
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| author | Mona S. El Ashry Enas Radwan Mona S. Abdellateif Omar Arafah Naglaa M. Hassan |
| author_facet | Mona S. El Ashry Enas Radwan Mona S. Abdellateif Omar Arafah Naglaa M. Hassan |
| author_sort | Mona S. El Ashry |
| collection | DOAJ |
| description | Abstract Background T-cell acute lymphoblastic leukemia (T-ALL) is an aggressive malignancy with few accepted prognostic factors that limit the efficiency of therapy. The aim of the current study was to assess the clinical and laboratory features of T-cell receptor (TCR) aberrations and early T-cell precursor (ETP) subtype as well as their outcome to therapy. Methods Sixty-three newly diagnosed pediatric T-ALL patients were assessed for the ETP status using immunophenotyping. Screening of TCRA/D aberrations was done by fluorescent in situ hybridization (FISH). The data were correlated to the patients’ clinical features, response to treatment, and survival rates. Results Seven patients (11%) had ETP-ALL. The ETP-ALL patients were older (P = 0.013), presented with lower white blood cell (WBC) count (P = 0.001) and lower percentage of peripheral blood (PB) blast cells (P = 0.037), more likely to have hyperdiploid karyotype (P = 0.009), and had been associated with TCRA/D gene amplification (P = 0.014) compared to other T-ALL patients. Of note, the same associations had been significantly observed in patients with TCRA/D gene amplification. Patients with TCRA/D amplification frequently coincided with TCRβ aberrations (P = 0.025). TCR-β aberrations were significantly associated with negative MRD at the end of induction compared to TCR-β-negative patients. There was a nonsignificant trend of ETP-positive cases to have lower overall survival (OS) (P = 0.06). Patients with TCR aberrations had no significant differences regarding disease-free survival (DFS) or OS rates compared to those with normal TCR. Conclusion ETP-ALL patients tend to have increased mortalities. There was no significant impact of TCR aberrations on the survival rates of the patients. |
| first_indexed | 2024-03-13T04:48:37Z |
| format | Article |
| id | doaj.art-9a5ec78ec6104c47a2a987874851219c |
| institution | Directory Open Access Journal |
| issn | 2589-0409 |
| language | English |
| last_indexed | 2024-03-13T04:48:37Z |
| publishDate | 2023-06-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | Journal of the Egyptian National Cancer Institute |
| spelling | doaj.art-9a5ec78ec6104c47a2a987874851219c2023-06-18T11:22:12ZengSpringerOpenJournal of the Egyptian National Cancer Institute2589-04092023-06-0135111210.1186/s43046-023-00176-1Clinical features, laboratory characteristics, and outcome of ETP and TCRA/D aberrations in pediatric patients with T-acute lymphoblastic leukemiaMona S. El Ashry0Enas Radwan1Mona S. Abdellateif2Omar Arafah3Naglaa M. Hassan4Clinical Pathology Department, National Cancer Institute, Cairo UniversityClinical Pathology Department, National Cancer Institute, Cairo UniversityMedical Biochemistry and Molecular Biology, Cancer Biology Department, National Cancer Institute, Cairo UniversityPediatric Oncology Department, National Cancer Institute, Cairo UniversityClinical Pathology Department, National Cancer Institute, Cairo UniversityAbstract Background T-cell acute lymphoblastic leukemia (T-ALL) is an aggressive malignancy with few accepted prognostic factors that limit the efficiency of therapy. The aim of the current study was to assess the clinical and laboratory features of T-cell receptor (TCR) aberrations and early T-cell precursor (ETP) subtype as well as their outcome to therapy. Methods Sixty-three newly diagnosed pediatric T-ALL patients were assessed for the ETP status using immunophenotyping. Screening of TCRA/D aberrations was done by fluorescent in situ hybridization (FISH). The data were correlated to the patients’ clinical features, response to treatment, and survival rates. Results Seven patients (11%) had ETP-ALL. The ETP-ALL patients were older (P = 0.013), presented with lower white blood cell (WBC) count (P = 0.001) and lower percentage of peripheral blood (PB) blast cells (P = 0.037), more likely to have hyperdiploid karyotype (P = 0.009), and had been associated with TCRA/D gene amplification (P = 0.014) compared to other T-ALL patients. Of note, the same associations had been significantly observed in patients with TCRA/D gene amplification. Patients with TCRA/D amplification frequently coincided with TCRβ aberrations (P = 0.025). TCR-β aberrations were significantly associated with negative MRD at the end of induction compared to TCR-β-negative patients. There was a nonsignificant trend of ETP-positive cases to have lower overall survival (OS) (P = 0.06). Patients with TCR aberrations had no significant differences regarding disease-free survival (DFS) or OS rates compared to those with normal TCR. Conclusion ETP-ALL patients tend to have increased mortalities. There was no significant impact of TCR aberrations on the survival rates of the patients.https://doi.org/10.1186/s43046-023-00176-1Early T-cell precursorChildT-ALLT-cell receptor |
| spellingShingle | Mona S. El Ashry Enas Radwan Mona S. Abdellateif Omar Arafah Naglaa M. Hassan Clinical features, laboratory characteristics, and outcome of ETP and TCRA/D aberrations in pediatric patients with T-acute lymphoblastic leukemia Journal of the Egyptian National Cancer Institute Early T-cell precursor Child T-ALL T-cell receptor |
| title | Clinical features, laboratory characteristics, and outcome of ETP and TCRA/D aberrations in pediatric patients with T-acute lymphoblastic leukemia |
| title_full | Clinical features, laboratory characteristics, and outcome of ETP and TCRA/D aberrations in pediatric patients with T-acute lymphoblastic leukemia |
| title_fullStr | Clinical features, laboratory characteristics, and outcome of ETP and TCRA/D aberrations in pediatric patients with T-acute lymphoblastic leukemia |
| title_full_unstemmed | Clinical features, laboratory characteristics, and outcome of ETP and TCRA/D aberrations in pediatric patients with T-acute lymphoblastic leukemia |
| title_short | Clinical features, laboratory characteristics, and outcome of ETP and TCRA/D aberrations in pediatric patients with T-acute lymphoblastic leukemia |
| title_sort | clinical features laboratory characteristics and outcome of etp and tcra d aberrations in pediatric patients with t acute lymphoblastic leukemia |
| topic | Early T-cell precursor Child T-ALL T-cell receptor |
| url | https://doi.org/10.1186/s43046-023-00176-1 |
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