Inside the biology of early T-cell precursor acute lymphoblastic leukemia: the perfect trick

• Published in: Biomarker research Vol. 9; no. 1; pp. 89 - 8
• Main Authors: Tarantini, Francesco, Cumbo, Cosimo, Anelli, Luisa, Zagaria, Antonella, Specchia, Giorgina, Musto, Pellegrino, Albano, Francesco
• Format: Journal Article
• Language: English
• Published: London BioMed Central 20.12.2021; BioMed Central Ltd; BMC
• Subjects: Acute lymphoblastic leukemia; Acute lymphocytic leukemia; Biology; Biomedical and Life Sciences; Biomedicine; Cancer; Cancer Research; Chemotherapy; Cytokines; ETP-ALL biology; Gene expression; Genomic instability; Genomics; Hypotheses; Kinases; Leukemia; Lymphatic leukemia; Lymphocytes T; Lymphoid-myeloid potential; Molecular pathogenesis; Mutation; Pediatrics; Precision medicine; Prognosis; Review; Stem cell leukemia; Stem cells; T cell receptors; T cells; Transcription factors; Lymphoid-myeloid potential; Molecular pathogenesis; ETP-ALL biology
• ISSN: 2050-7771; 2050-7771
• DOI: 10.1186/s40364-021-00347-z

Early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is a rare, distinct subtype of T-ALL characterized by genomic instability, a dismal prognosis and refractoriness to standard chemotherapy. Since its first description in 2009, the expanding knowledge of its intricate biology has led to the definition of a stem cell leukemia with a combined lymphoid-myeloid potential: the perfect trick. Several studies in the last decade aimed to better characterize this new disease, but it was recognized as a distinct entity only in 2016. We review current insights into the biology of ETP-ALL and discuss the pathogenesis, genomic features and their impact on the clinical course in the precision medicine era today.