T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management
T-cell acute lymphoblastic leukemia (T-ALL) is an uncommon, yet aggressive leukemia that accounts for approximately one-fourth of acute lymphoblastic leukemia (ALL) cases. <i>CDKN2A</i>/<i>CDKN2B</i> and <i>NOTCH1</i> are the most common mutated genes in T-ALL. Ch...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-11-01
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| Series: | Biomedicines |
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| Online Access: | https://www.mdpi.com/2227-9059/9/11/1621 |
| _version_ | 1797511169533018112 |
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| author | Parveen Shiraz Waqas Jehangir Vaibhav Agrawal |
| author_facet | Parveen Shiraz Waqas Jehangir Vaibhav Agrawal |
| author_sort | Parveen Shiraz |
| collection | DOAJ |
| description | T-cell acute lymphoblastic leukemia (T-ALL) is an uncommon, yet aggressive leukemia that accounts for approximately one-fourth of acute lymphoblastic leukemia (ALL) cases. <i>CDKN2A</i>/<i>CDKN2B</i> and <i>NOTCH1</i> are the most common mutated genes in T-ALL. Children and young adults are treated with pediatric intensive regimens and have superior outcomes compared to older adults. In children and young adults, Nelarabine added to frontline chemotherapy improves outcomes and end of consolidation measurable residual disease has emerged as the most valuable prognostic marker. While outcomes for de-novo disease are steadily improving, patients with relapsed and refractory T-ALL fare poorly. Newer targeted therapies are being studied in large clinical trials and have the potential to further improve outcomes. The role of allogeneic stem cell transplant (HSCT) is evolving due to the increased use of pediatric-inspired regimens and MRD monitoring. In this review we will discuss the biology, treatment, and outcomes in pediatric and adult T-ALL. |
| first_indexed | 2024-03-10T05:41:38Z |
| format | Article |
| id | doaj.art-cbf3dcaa43fb42edb04875642153b60d |
| institution | Directory Open Access Journal |
| issn | 2227-9059 |
| language | English |
| last_indexed | 2024-03-10T05:41:38Z |
| publishDate | 2021-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomedicines |
| spelling | doaj.art-cbf3dcaa43fb42edb04875642153b60d2023-11-22T22:30:55ZengMDPI AGBiomedicines2227-90592021-11-01911162110.3390/biomedicines9111621T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and ManagementParveen Shiraz0Waqas Jehangir1Vaibhav Agrawal2Blood and Marrow Transplantation/Cell Therapy, Stanford University, Stanford, CA 94305, USAAvera Medical Group Hematology, Transplant & Cellular Therapy, Sioux Falls, SD 57105, USADepartment of Hematology & Hematopoietic Cell Transplantation, City of Hope National Medical Center, Duarte, CA 91010, USAT-cell acute lymphoblastic leukemia (T-ALL) is an uncommon, yet aggressive leukemia that accounts for approximately one-fourth of acute lymphoblastic leukemia (ALL) cases. <i>CDKN2A</i>/<i>CDKN2B</i> and <i>NOTCH1</i> are the most common mutated genes in T-ALL. Children and young adults are treated with pediatric intensive regimens and have superior outcomes compared to older adults. In children and young adults, Nelarabine added to frontline chemotherapy improves outcomes and end of consolidation measurable residual disease has emerged as the most valuable prognostic marker. While outcomes for de-novo disease are steadily improving, patients with relapsed and refractory T-ALL fare poorly. Newer targeted therapies are being studied in large clinical trials and have the potential to further improve outcomes. The role of allogeneic stem cell transplant (HSCT) is evolving due to the increased use of pediatric-inspired regimens and MRD monitoring. In this review we will discuss the biology, treatment, and outcomes in pediatric and adult T-ALL.https://www.mdpi.com/2227-9059/9/11/1621acute lymphoblastic leukemiaT-cell leukemiaNOTCHNelarabine |
| spellingShingle | Parveen Shiraz Waqas Jehangir Vaibhav Agrawal T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management Biomedicines acute lymphoblastic leukemia T-cell leukemia NOTCH Nelarabine |
| title | T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management |
| title_full | T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management |
| title_fullStr | T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management |
| title_full_unstemmed | T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management |
| title_short | T-Cell Acute Lymphoblastic Leukemia—Current Concepts in Molecular Biology and Management |
| title_sort | t cell acute lymphoblastic leukemia current concepts in molecular biology and management |
| topic | acute lymphoblastic leukemia T-cell leukemia NOTCH Nelarabine |
| url | https://www.mdpi.com/2227-9059/9/11/1621 |
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