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

David I. Marks, Elisabeth M. Paietta, Anthony V. Moorman, Susan M. Richards, Georgina Buck, Gordon DeWald, Adolfo Ferrando, Adele K. Fielding, Anthony H. Goldstone, Rhett P. Ketterling, Mark R. Litzow, Selina M. Luger, Andrew K. McMillan, Marc R. Mansour, Jacob M. Rowe, Martin S. Tallman, Hillard M. Lazarus

Research output: Contribution to journalArticle

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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%, 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.

Original languageEnglish (US)
Pages (from-to)5136-5145
Number of pages10
JournalBlood
Volume114
Issue number25
DOIs
StatePublished - Dec 10 2009

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T-cells
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Cytogenetics
T-Lymphocytes
Chemotherapy
Autografts
Neurology
Survival
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma
Tissue Donors
Pharmaceutical Preparations
Chromosome Aberrations
Siblings
Arm
Central Nervous System
Recurrence
Drug Therapy
Mutation
nelarabine

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology

Cite this

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

In: Blood, Vol. 114, No. 25, 10.12.2009, p. 5136-5145.

Research output: Contribution to journalArticle

Marks, DI, Paietta, EM, Moorman, AV, Richards, SM, Buck, G, DeWald, G, Ferrando, A, Fielding, AK, Goldstone, AH, Ketterling, RP, Litzow, MR, Luger, SM, McMillan, AK, Mansour, MR, Rowe, JM, Tallman, MS & Lazarus, HM 2009, 'T-cell acute lymphoblastic leukemia in adults: Clinical features, immunophenotype, cytogenetics, and outcome from the large randomized prospective trial (UKALL XII/ECOG 2993)', Blood, vol. 114, no. 25, pp. 5136-5145. https://doi.org/10.1182/blood-2009-08-231217
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. / T-cell acute lymphoblastic leukemia in adults : Clinical features, immunophenotype, cytogenetics, and outcome from the large randomized prospective trial (UKALL XII/ECOG 2993). In: Blood. 2009 ; Vol. 114, No. 25. pp. 5136-5145.
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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{\%}, 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.",
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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.

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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.

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