TY - JOUR AU - David I. Marks, AU - Anthony V. Moorman, AU - Lucy Chilton, AU - Elisabeth Paietta, AU - Amir Enshaie, AU - Gordon DeWald, AU - Christine J. Harrison, AU - Adele K. Fielding, AU - Letizia Foroni, AU - Anthony H. Goldstone, AU - Mark R. Litzow, AU - Selina M. Luger, AU - Andrew K. McMillan, AU - Janis Racevskis, AU - Jacob M. Rowe, AU - Martin S. Tallman, AU - Peter Wiernik, AU - Hillard M. Lazarus, PY - 2013/05/31 Y2 - 2024/03/28 TI - 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 JF - Haematologica JA - haematol VL - 98 IS - 6 SE - Articles DO - 10.3324/haematol.2012.081877 UR - https://haematologica.org/article/view/6697 SP - 945-952 AB - 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