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Burkitt lymphoma: bridging the gap between advances in molecular biology and therapy
- Source :
- Leuk Lymphoma
- Publication Year :
- 2020
- Publisher :
- Informa UK Limited, 2020.
-
Abstract
- Genomic studies have revealed molecular mechanisms involved in the pathogenesis of Burkitt’s lymphoma, including the ID3/TCF3-dependent centroblast gene expression program, tonic PI3K-AKT-mTOR signaling, and deregulation of cell cycle and apoptosis through mutations in cyclin D3, CDKN2A, or TP53. Unfortunately, these advances have not been translated into treatment, which relies on dose-intense cytotoxic chemotherapy. While most patients achieve long-term survival, options for relapsed/refractory disease are lacking, as Burkitt lymphoma is often excluded from clinical trials of novel approaches. The lower-intensity, dose-adjusted EPOCH plus rituximab (DA-EPOCH-R) regimen constitutes a major advance allowing for treatment of older and HIV-positive patients but needs augmentation to better address the central nervous system involvement. Furthermore, DA-EPOCH-R provides a platform for the study of targeted or immunotherapeutic approaches while de-escalating cytotoxic agents and their associated adverse effects. In this review we discuss the epidemiology and molecular genetics of BL, first-line treatment considerations, and potential novel treatment strategies.
- Subjects :
- Cancer Research
medicine.medical_specialty
medicine.medical_treatment
Article
Phosphatidylinositol 3-Kinases
03 medical and health sciences
0302 clinical medicine
CDKN2A
Molecular genetics
Antineoplastic Combined Chemotherapy Protocols
medicine
Humans
Molecular Biology
Chemotherapy
business.industry
Hematology
Immunotherapy
Cell cycle
medicine.disease
Burkitt Lymphoma
Lymphoma
Regimen
Oncology
030220 oncology & carcinogenesis
Cancer research
Rituximab
business
Signal Transduction
030215 immunology
medicine.drug
Subjects
Details
- ISSN :
- 10292403 and 10428194
- Volume :
- 61
- Database :
- OpenAIRE
- Journal :
- Leukemia & Lymphoma
- Accession number :
- edsair.doi.dedup.....782d705dc4550d2d6557abcaae12f65b