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Blood, 10 December 2009, Vol. 114, No. 25, pp. 5136-5145.
Prepublished online as a Blood First Edition Paper on October 14, 2009; DOI 10.1182/blood-2009-08-231217.


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Submitted August 4, 2009; accepted September 21, 2009.

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

David I. Marks1,13, Elisabeth M. Paietta2, Anthony V. Moorman3, Susan M. Richards4, Georgina Buck4, Gordon DeWald5, Adolfo Ferrando6, Adele K. Fielding7, Anthony H Goldstone7, Rhett P. Ketterling5, Mark R. Litzow5, Selina M. Luger8, Andrew K. McMillan9, Marc Mansour7, Jacob M. Rowe10, Martin S. Tallman11 and Hillard M. Lazarus12

1 University Hospitals Bristol NHS Foundation Trust, United Kingdom; 2 Montefiore Medical Center, United States; 3 Newcastle University, United Kingdom; 4 CTSU, United Kingdom; 5 Mayo Clinic, United Kingdom; 6 Columbia University, United States; 7 University College London, United States; 8 University of Pennsylvania, United Kingdom; 9 Nottingham City Hospital, United Kingdom; 10 Rambam Medical Center, United States; 11 Northwestern University, United States; 12 Case Western University, United States

* Corresponding author; email: david.marks{at}ubht.nhs.uk

Abstract

The biology and outcome of adult T-cell acute lymphoblastic leukemia are poorly understood. We present here the clinical and biological 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=0.01 and 0.0005 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=0.006). Central nervous system involvement at diagnosis did not affect survival (47% vs 48%, p=NS). For 99 patients randomised 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-square p=0.02); this was due to less relapse (25% vs 51% at 5 years, p<0.001). 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.


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