Comprehensive Overview of Gene Rearrangements in Childhood T-Cell Acute Lymphoblastic Leukaemia
Acute lymphoblastic leukaemia (ALL) is a relevant form of childhood neoplasm, as it accounts for over 80% of all leukaemia cases. T-cell ALL constitutes a genetically heterogeneous cancer derived from T-lymphoid progenitors. The diagnosis of T-ALL is based on morphologic, immunophenotypic, cytogenet...
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doaj-2a7934fb38c74593b38a82d2555646d72021-01-16T00:00:37ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672021-01-012280880810.3390/ijms22020808Comprehensive Overview of Gene Rearrangements in Childhood T-Cell Acute Lymphoblastic LeukaemiaAnna Mroczek0Joanna Zawitkowska1Jerzy Kowalczyk2Monika Lejman3Department of Paediatric Haematology, Oncology and Transplantology, Medical University of Lublin, 20-093 Lublin, PolandDepartment of Paediatric Haematology, Oncology and Transplantology, Medical University of Lublin, 20-093 Lublin, PolandDepartment of Paediatric Haematology, Oncology and Transplantology, Medical University of Lublin, 20-093 Lublin, PolandLaboratory of Genetic Diagnostics, Medical University of Lublin, 20-093 Lublin, PolandAcute lymphoblastic leukaemia (ALL) is a relevant form of childhood neoplasm, as it accounts for over 80% of all leukaemia cases. T-cell ALL constitutes a genetically heterogeneous cancer derived from T-lymphoid progenitors. The diagnosis of T-ALL is based on morphologic, immunophenotypic, cytogenetic, and molecular features, thus the results are used for patient stratification. Due to the expression of surface and intracellular antigens, several subtypes of T-ALL can be distinguished. Although the aetiology of T-ALL remains unclear, a wide spectrum of rearrangements and mutations affecting crucial signalling pathways has been described so far. Due to intensive chemotherapy regimens and supportive care, overall cure rates of more than 80% in paediatric T-ALL patients have been accomplished. However, improved knowledge of the mechanisms of relapse, drug resistance, and determination of risk factors are crucial for patients in the high-risk group. Even though some residual disease studies have allowed the optimization of therapy, the identification of novel diagnostic and prognostic markers is required to individualize therapy. The following review summarizes our current knowledge about genetic abnormalities in paediatric patients with T-ALL. As molecular biology techniques provide insights into the biology of cancer, our study focuses on new potential therapeutic targets and predictive factors which may improve the outcome of young patients with T-ALL.https://www.mdpi.com/1422-0067/22/2/808T-ALLgenomic landscapepaediatrics |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Anna Mroczek Joanna Zawitkowska Jerzy Kowalczyk Monika Lejman |
spellingShingle |
Anna Mroczek Joanna Zawitkowska Jerzy Kowalczyk Monika Lejman Comprehensive Overview of Gene Rearrangements in Childhood T-Cell Acute Lymphoblastic Leukaemia International Journal of Molecular Sciences T-ALL genomic landscape paediatrics |
author_facet |
Anna Mroczek Joanna Zawitkowska Jerzy Kowalczyk Monika Lejman |
author_sort |
Anna Mroczek |
title |
Comprehensive Overview of Gene Rearrangements in Childhood T-Cell Acute Lymphoblastic Leukaemia |
title_short |
Comprehensive Overview of Gene Rearrangements in Childhood T-Cell Acute Lymphoblastic Leukaemia |
title_full |
Comprehensive Overview of Gene Rearrangements in Childhood T-Cell Acute Lymphoblastic Leukaemia |
title_fullStr |
Comprehensive Overview of Gene Rearrangements in Childhood T-Cell Acute Lymphoblastic Leukaemia |
title_full_unstemmed |
Comprehensive Overview of Gene Rearrangements in Childhood T-Cell Acute Lymphoblastic Leukaemia |
title_sort |
comprehensive overview of gene rearrangements in childhood t-cell acute lymphoblastic leukaemia |
publisher |
MDPI AG |
series |
International Journal of Molecular Sciences |
issn |
1661-6596 1422-0067 |
publishDate |
2021-01-01 |
description |
Acute lymphoblastic leukaemia (ALL) is a relevant form of childhood neoplasm, as it accounts for over 80% of all leukaemia cases. T-cell ALL constitutes a genetically heterogeneous cancer derived from T-lymphoid progenitors. The diagnosis of T-ALL is based on morphologic, immunophenotypic, cytogenetic, and molecular features, thus the results are used for patient stratification. Due to the expression of surface and intracellular antigens, several subtypes of T-ALL can be distinguished. Although the aetiology of T-ALL remains unclear, a wide spectrum of rearrangements and mutations affecting crucial signalling pathways has been described so far. Due to intensive chemotherapy regimens and supportive care, overall cure rates of more than 80% in paediatric T-ALL patients have been accomplished. However, improved knowledge of the mechanisms of relapse, drug resistance, and determination of risk factors are crucial for patients in the high-risk group. Even though some residual disease studies have allowed the optimization of therapy, the identification of novel diagnostic and prognostic markers is required to individualize therapy. The following review summarizes our current knowledge about genetic abnormalities in paediatric patients with T-ALL. As molecular biology techniques provide insights into the biology of cancer, our study focuses on new potential therapeutic targets and predictive factors which may improve the outcome of young patients with T-ALL. |
topic |
T-ALL genomic landscape paediatrics |
url |
https://www.mdpi.com/1422-0067/22/2/808 |
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