TY - JOUR
T1 - T-cell acute lymphoblastic leukemia in adults
T2 - Clinical features, immunophenotype, cytogenetics, and outcome from the large randomized prospective trial (UKALL XII/ECOG 2993)
AU - Marks, David I.
AU - Paietta, Elisabeth M.
AU - Moorman, Anthony V.
AU - Richards, Susan M.
AU - Buck, Georgina
AU - DeWald, Gordon
AU - Ferrando, Adolfo
AU - Fielding, Adele K.
AU - Goldstone, Anthony H.
AU - Ketterling, Rhett P.
AU - Litzow, Mark R.
AU - Luger, Selina M.
AU - McMillan, Andrew K.
AU - Mansour, Marc R.
AU - Rowe, Jacob M.
AU - Tallman, Martin S.
AU - Lazarus, Hillard M.
PY - 2009/12/10
Y1 - 2009/12/10
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=73949110789&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=73949110789&partnerID=8YFLogxK
U2 - 10.1182/blood-2009-08-231217
DO - 10.1182/blood-2009-08-231217
M3 - Article
C2 - 19828704
AN - SCOPUS:73949110789
SN - 0006-4971
VL - 114
SP - 5136
EP - 5145
JO - Blood
JF - Blood
IS - 25
ER -