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

Author:

Marks David I.1,Paietta Elisabeth M.2,Moorman Anthony V.3,Richards Susan M.4,Buck Georgina4,DeWald Gordon5,Ferrando Adolfo6,Fielding Adele K.7,Goldstone Anthony H.7,Ketterling Rhett P.5,Litzow Mark R.5,Luger Selina M.8,McMillan Andrew K.9,Mansour Marc R.7,Rowe Jacob M.10,Tallman Martin S.11,Lazarus Hillard M.12

Affiliation:

1. Adult Bone Marrow Transplant Unit, University Hospitals Bristol National Health Service Foundation Trust, Bristol, United Kingdom;

2. Montefiore Medical Center, New York, NY;

3. Leukaemia Research Cytogenetics Group, Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne, United Kingdom;

4. Clinical Trial Service Unit, Oxford, United Kingdom;

5. Mayo Clinic, Rochester, MN;

6. Columbia University, New York, NY;

7. University College London, London, United Kingdom;

8. University of Pennsylvania, Philadelphia;

9. Nottingham City Hospital, Nottingham, United Kingdom;

10. Rambam Medical Center, Haifa, Israel;

11. Northwestern University, Chicago, IL; and

12. Case Western Reserve University, Cleveland, OH

Abstract

AbstractThe 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.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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