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
Affiliations
David I. Marks
Adult BMT Unit, University Hospitals Bristol NHS Foundation Trust, Bristol, UK
Anthony V. Moorman
Leukaemia Research Cytogenetics Group, Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne, UK
Lucy Chilton
Leukaemia Research Cytogenetics Group, Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne, UK
Elisabeth Paietta
Montefiore Medical Center, New York, NY, USA
Amir Enshaie
Leukaemia Research Cytogenetics Group, Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne, UK
Gordon DeWald
Mayo Clinic, Rochester MN, USA
Christine J. Harrison
Leukaemia Research Cytogenetics Group, Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne, UK
Adele K. Fielding
University College London, London, UK
Letizia Foroni
Imperial College, London, UK
Anthony H. Goldstone
University College London, London, UK
Mark R. Litzow
Mayo Clinic, Rochester MN, USA
Selina M. Luger
University of Pennysylvania, Philadelphia, PA, USA
Andrew K. McMillan
Nottingham City Hospital, Nottingham, UK
Janis Racevskis
Montefiore Medical Center, New York, NY, USA
Jacob M. Rowe
Shaare Zedek Medical Center, Jerusalem, Israel
Martin S. Tallman
Memorial Sloan-Kettering Cancer Center, New York, NY, USA
Peter Wiernik
Montefiore Medical Center, New York, NY, USA
Hillard M. Lazarus
Case Western Reserve University, University Hospitals Case Medical Center, Cleveland OH, USA
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
