KMT2A-rearranged acute lymphoblastic leukaemia
KMT2A-rearranged acute lymphoblastic leukaemia (ALL) represents a high risk subtype of childhood ALL. Historical treatment strategies have comprised of intensification with conventional chemotherapy. However, outcomes have remained consistently poor compared to the advances that have been seen for o...
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| Format: | Article |
| Language: | English |
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Elsevier
2024-12-01
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| Series: | EJC Paediatric Oncology |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2772610X24000643 |
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| author | Rishi S. Kotecha Rob Pieters Janine Stutterheim |
| author_facet | Rishi S. Kotecha Rob Pieters Janine Stutterheim |
| author_sort | Rishi S. Kotecha |
| collection | DOAJ |
| description | KMT2A-rearranged acute lymphoblastic leukaemia (ALL) represents a high risk subtype of childhood ALL. Historical treatment strategies have comprised of intensification with conventional chemotherapy. However, outcomes have remained consistently poor compared to the advances that have been seen for other ALL subtypes, particularly for infants diagnosed before their first birthday. The advent of novel immunotherapeutic approaches has led to a change in the treatment paradigm, with the integration of blinatumomab to the current suite of clinical trials for KMT2A-rearranged ALL expected to result in marked improvements. Furthermore, significant progress has been made to understand the unique biology of KMT2A-rearranged ALL, which has led to the development of novel agents that directly target the KMT2A complex or dysregulated proteins/pathways. Clinical trials are currently poised to evaluate therapies such as venetoclax and menin inhibitors, offering further hope for achieving a cure. In this review, we discuss the remarkable progress that has been made for KMT2A-rearranged ALL, leading to much optimism for improved outcomes in the future. |
| format | Article |
| id | doaj-art-ed77b18b8df94a55ae1556f991d6f64c |
| institution | Kabale University |
| issn | 2772-610X |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | EJC Paediatric Oncology |
| spelling | doaj-art-ed77b18b8df94a55ae1556f991d6f64c2024-12-13T11:08:34ZengElsevierEJC Paediatric Oncology2772-610X2024-12-014100204KMT2A-rearranged acute lymphoblastic leukaemiaRishi S. Kotecha0Rob Pieters1Janine Stutterheim2Department of Clinical Haematology, Oncology, Blood and Marrow Transplantation, Perth Children’s Hospital, Perth, Australia; Leukaemia Translational Research Laboratory, Telethon Kids Cancer Centre, Telethon Kids Institute, University of Western Australia, Perth, Australia; Curtin Medical School, Curtin University, Perth, Australia; Correspondence to: Perth Children’s Hospital, Level 2D, 15 Hospital Avenue, Nedlands, Perth 6009, Australia.Princess Maxima Center for Pediatric Oncology, Utrecht, NetherlandsPrincess Maxima Center for Pediatric Oncology, Utrecht, Netherlands; Correspondence to: Princess Maxima Center, Heidelberglaan 25, CS Utrecht 3435, Netherlands.KMT2A-rearranged acute lymphoblastic leukaemia (ALL) represents a high risk subtype of childhood ALL. Historical treatment strategies have comprised of intensification with conventional chemotherapy. However, outcomes have remained consistently poor compared to the advances that have been seen for other ALL subtypes, particularly for infants diagnosed before their first birthday. The advent of novel immunotherapeutic approaches has led to a change in the treatment paradigm, with the integration of blinatumomab to the current suite of clinical trials for KMT2A-rearranged ALL expected to result in marked improvements. Furthermore, significant progress has been made to understand the unique biology of KMT2A-rearranged ALL, which has led to the development of novel agents that directly target the KMT2A complex or dysregulated proteins/pathways. Clinical trials are currently poised to evaluate therapies such as venetoclax and menin inhibitors, offering further hope for achieving a cure. In this review, we discuss the remarkable progress that has been made for KMT2A-rearranged ALL, leading to much optimism for improved outcomes in the future.http://www.sciencedirect.com/science/article/pii/S2772610X24000643Acute lymphoblastic leukaemiaKMT2A-rearrangedInfantPaediatric |
| spellingShingle | Rishi S. Kotecha Rob Pieters Janine Stutterheim KMT2A-rearranged acute lymphoblastic leukaemia EJC Paediatric Oncology Acute lymphoblastic leukaemia KMT2A-rearranged Infant Paediatric |
| title | KMT2A-rearranged acute lymphoblastic leukaemia |
| title_full | KMT2A-rearranged acute lymphoblastic leukaemia |
| title_fullStr | KMT2A-rearranged acute lymphoblastic leukaemia |
| title_full_unstemmed | KMT2A-rearranged acute lymphoblastic leukaemia |
| title_short | KMT2A-rearranged acute lymphoblastic leukaemia |
| title_sort | kmt2a rearranged acute lymphoblastic leukaemia |
| topic | Acute lymphoblastic leukaemia KMT2A-rearranged Infant Paediatric |
| url | http://www.sciencedirect.com/science/article/pii/S2772610X24000643 |
| work_keys_str_mv | AT rishiskotecha kmt2arearrangedacutelymphoblasticleukaemia AT robpieters kmt2arearrangedacutelymphoblasticleukaemia AT janinestutterheim kmt2arearrangedacutelymphoblasticleukaemia |