title: T-cell acute lymphoblastic leukemia in adults: clinical features, immunophenotype, cytogenetics, and outcome from the large randomized prospective trial (UKALL XII/ECOG 2993).
abstract: The biology and outcome of adult T-cell acute lymphoblastic leukemia are poorly understood. We present here the clinical and biologic features of 356 patients treated uniformly on the prospective trial (UKALL XII/ECOG 2993) with the aim of describing the outcome and identifying prognostic factors. Complete remission was obtained in 94% of patients, and 48% survived 5 years. Positivity of blasts for CD1a and lack of expression of CD13 were associated with better survival (P = .01 and < .001, respectively). NOTCH1 and CDKN2A mutations were seen in 61% and 42% of those tested. Complex cytogenetic abnormalities were associated with poorer survival (19% vs 51% at 5 years, P = .006). Central nervous system involvement at diagnosis did not affect survival (47% vs 48%, P = not significant). For 99 patients randomized between autograft and chemotherapy, 5-year survival was 51% in each arm. Patients with a matched sibling donor had superior 5-year survival to those without donors (61% vs 46%, chi(2), P = .02); this was the result of less relapse (25% vs 51% at 5 years, P < .001). Only 8 of 123 relapsed patients survive. This study provides a baseline for trials of new drugs, such as nelarabine, and may allow risk-adapted therapy in patients with poor-prognosis T-cell ALL.
authors.affiliation: Adult Bone Marrow Transplant Unit, University Hospitals Bristol National Health Service Foundation Trust, Bristol, United Kingdom. David.Marks@ubht.nhs.uk
authors.forename: David I
authors.initials: DI
authors.lastname: Marks
authors.forename: Elisabeth M
authors.initials: EM
authors.lastname: Paietta
authors.forename: Anthony V
authors.initials: AV
authors.lastname: Moorman
authors.forename: Susan M
authors.initials: SM
authors.lastname: Richards
authors.forename: Georgina
authors.initials: G
authors.lastname: Buck
authors.forename: Gordon
authors.initials: G
authors.lastname: DeWald
authors.forename: Adolfo
authors.initials: A
authors.lastname: Ferrando
authors.forename: Adele K
authors.initials: AK
authors.lastname: Fielding
authors.forename: Anthony H
authors.initials: AH
authors.lastname: Goldstone
authors.forename: Rhett P
authors.initials: RP
authors.lastname: Ketterling
authors.forename: Mark R
authors.initials: MR
authors.lastname: Litzow
authors.forename: Selina M
authors.initials: SM
authors.lastname: Luger
authors.forename: Andrew K
authors.initials: AK
authors.lastname: McMillan
authors.forename: Marc R
authors.initials: MR
authors.lastname: Mansour
authors.forename: Jacob M
authors.initials: JM
authors.lastname: Rowe
authors.forename: Martin S
authors.initials: MS
authors.lastname: Tallman
authors.forename: Hillard M
authors.initials: HM
authors.lastname: Lazarus
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D020732:Cytogenetic Analysis; D005260:Female; D016044:Fusion Proteins, bcr-abl / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D008875:Middle Aged; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D011446:Prospective Studies; D012074:Remission Induction; D033581:Stem Cell Transplantation / Q000379:methods*; D016019:Survival Analysis; D013601:T-Lymphocytes / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D016044:Fusion Proteins, bcr-abl