title: Molecular pathogenesis of T-cell leukaemia and lymphoma.
abstract: T-cell acute lymphoblastic leukaemia (T-ALL) is induced by the transformation of T-cell progenitors and mainly occurs in children and adolescents. Although treatment outcome in patients with T-ALL has improved in recent years, patients with relapsed disease continue to have a poor prognosis. It is therefore important to understand the molecular pathways that control both the induction of transformation and the treatment of relapsed disease. In this Review, we focus on the molecular mechanisms responsible for disease induction and maintenance. We also compare the physiological progression of T-cell differentiation with T-cell transformation, highlighting the close relationship between these two processes. Finally, we discuss potential new therapies that target oncogenic pathways in T-ALL.
authors.affiliation: Department of Pathology, New York University School of Medicine, 550 First Avenue, New York, New York 10016, USA. iannis.aifantis@med.nyu.edu
authors.forename: Iannis
authors.initials: I
authors.lastname: Aifantis
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Raetz
authors.forename: Silvia
authors.initials: S
authors.lastname: Buonamici
mesh_terms: D000818:Animals; D051792:Basic Helix-Loop-Helix Proteins / Q000378:metabolism; D018797:Cell Cycle Proteins / Q000235:genetics; D002454:Cell Differentiation; D019070:Cell Lineage; D002648:Child; D044783:F-Box Proteins / Q000235:genetics; D000073979:F-Box-WD Repeat-Containing Protein 7; D015973:Gene Expression Regulation, Leukemic; D018398:Homeodomain Proteins / Q000378:metabolism; D006801:Humans; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D008223:Lymphoma / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D051379:Mice; D009154:Mutation; D016328:NF-kappa B / Q000378:metabolism; D051881:Receptor, Notch1 / Q000235:genetics / Q000378:metabolism; D015398:Signal Transduction; D013601:T-Lymphocytes / Q000166:cytology / Q000378:metabolism*; D013950:Thymus Gland / Q000254:growth & development / Q000276:immunology; D014157:Transcription Factors / Q000378:metabolism; D014178:Translocation, Genetic; D044767:Ubiquitin-Protein Ligases / Q000235:genetics; D054875:Ubiquitination
chemical_list: D051792:Basic Helix-Loop-Helix Proteins; D018797:Cell Cycle Proteins; D044783:F-Box Proteins; D000073979:F-Box-WD Repeat-Containing Protein 7; D018398:Homeodomain Proteins; D016328:NF-kappa B; D051881:Receptor, Notch1; D014157:Transcription Factors; D044767:Ubiquitin-Protein Ligases; C513273:FBXW7 protein, human