T-cell acute lymphoblastic leukemia in adults: clinical features, immunophenotype, cytogenetics, and outcome from the large randomized prospective trial (UKALL XII/ECOG 2993)

• Published in: Blood Vol. 114; no. 25; pp. 5136 - 5145
• Main Authors: Marks, David I., Paietta, Elisabeth M., Moorman, Anthony V., Richards, Susan M., Buck, Georgina, DeWald, Gordon, Ferrando, Adolfo, Fielding, Adele K., Goldstone, Anthony H., Ketterling, Rhett P., Litzow, Mark R., Luger, Selina M., McMillan, Andrew K., Mansour, Marc R., Rowe, Jacob M., Tallman, Martin S., Lazarus, Hillard M.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 10.12.2009; American Society of Hematology
• Series: Clinical Trials and Observations
• Subjects: Adult; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Clinical Trials and Observations; Cytogenetic Analysis; Female; Fusion Proteins, bcr-abl - genetics; Humans; Immunophenotyping; In Situ Hybridization, Fluorescence; Male; Middle Aged; Precursor Cell Lymphoblastic Leukemia-Lymphoma - genetics; Precursor Cell Lymphoblastic Leukemia-Lymphoma - pathology; Precursor Cell Lymphoblastic Leukemia-Lymphoma - therapy; Prospective Studies; Remission Induction; Stem Cell Transplantation - methods; Survival Analysis; T-Lymphocytes - immunology; T-Lymphocytes - metabolism; T-Lymphocytes - pathology; Transplantation, Homologous; Treatment Outcome; Young Adult
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2009-08-231217

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%, χ2, 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.