Haematologica (Jun 2013)

The clinical characteristics, therapy and outcome of 85 adults with acute lymphoblastic leukemia and t(4;11)(q21;q23)/MLL-AFF1 prospectively treated in the UKALLXII/ECOG2993 trial

  • David I. Marks,
  • Anthony V. Moorman,
  • Lucy Chilton,
  • Elisabeth Paietta,
  • Amir Enshaie,
  • Gordon DeWald,
  • Christine J. Harrison,
  • Adele K. Fielding,
  • Letizia Foroni,
  • Anthony H. Goldstone,
  • Mark R. Litzow,
  • Selina M. Luger,
  • Andrew K. McMillan,
  • Janis Racevskis,
  • Jacob M. Rowe,
  • Martin S. Tallman,
  • Peter Wiernik,
  • Hillard M. Lazarus

DOI
https://doi.org/10.3324/haematol.2012.081877
Journal volume & issue
Vol. 98, no. 6

Abstract

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The biology and outcome of adult t(4;11)(q21;q23)/MLL-AFF1 acute lymphoblastic leukemia are poorly understood. We describe the outcome and delineate prognostic factors and optimal post-remission therapy in 85 consecutive patients (median age 38 years) treated uniformly in the prospective trial UKALLXII/ECOG2993. The immunophenotype of this leukemia was pro-B (CD10NEG). Immaturity was further suggested by high expression of the stem-cell antigens, CD133 and CD135, although CD34 expression was significantly lower than in t(4;11)-negative patients. Complete remission was achieved in 77 (93%) patients but only 35% survived 5 years (95% CI: 25–45%); the relapse rate was 45% (95% CI: 33–58%). Thirty-one patients underwent allogeneic transplantation in first remission (15 sibling donors and 16 unrelated donors): with 5-year survival rates of 56% and 67% respectively, only 2/31 patients relapsed. This compares with a 24% survival rate and 59% relapse rate in 46 patients who received post-remission chemotherapy. A major determinant of outcome was age with 71% of patients aged