PURPOSE: To determine the molecular characteristics, clinical features, and treatment outcomes of children with acute lymphoblastic leukemia (ALL) and the t(11;19)(q23,p13.3) translocation. PATIENTS AND METHODS: A retrospective analysis of leukemic cell karyotypes obtained from patients with new diagnoses of ALL who were treated at St. Jude Children's Research Hospital or by the Pediatric Oncology Group was performed to identify cases with the t(11;19)(q23;p13.3) translocation. Molecular analyses were performed on these cases to determine the status of the MLL gene and the presence of the MLL-ENL fusion transcript. RESULTS: Among 3,578 patients with ALL and successful cytogenetic analysis, we identified 35 patients with the t(11;19)(q23;p13.3) translocation: 13 infants and 11 older children had B-precursor leukemia, whereas 11 patients had a T-cell phenotype. Although all of the cases examined had MLL rearrangements and MLL-ENL fusion transcripts, outcome varied according to age and immunophenotype. Among B-precursor cases, only two of the 13 infants remain in complete remission, compared with six of the 11 older children. Most strikingly, no relapses have occurred among B-precursor patients 1 to 9 years of age or among T-cell patients. CONCLUSION: Although MLL gene rearrangements are generally associated with a dismal outcome in ALL, two distinct subsets with MLL-ENL fusions have an excellent prognosis. Our results suggest that patients with this genetic abnormality who have T-cell ALL or are 1 to 9 years of age should not be considered candidates for hematopoietic stem-cell transplantation during their first remission.
PURPOSE: To determine the molecular characteristics, clinical features, and treatment outcomes of children with acute lymphoblastic leukemia (ALL) and the t(11;19)(q23,p13.3) translocation. PATIENTS AND METHODS: A retrospective analysis of leukemic cell karyotypes obtained from patients with new diagnoses of ALL who were treated at St. Jude Children's Research Hospital or by the Pediatric Oncology Group was performed to identify cases with the t(11;19)(q23;p13.3) translocation. Molecular analyses were performed on these cases to determine the status of the MLL gene and the presence of the MLL-ENL fusion transcript. RESULTS: Among 3,578 patients with ALL and successful cytogenetic analysis, we identified 35 patients with the t(11;19)(q23;p13.3) translocation: 13 infants and 11 older children had B-precursor leukemia, whereas 11 patients had a T-cell phenotype. Although all of the cases examined had MLL rearrangements and MLL-ENL fusion transcripts, outcome varied according to age and immunophenotype. Among B-precursor cases, only two of the 13 infants remain in complete remission, compared with six of the 11 older children. Most strikingly, no relapses have occurred among B-precursor patients 1 to 9 years of age or among T-cell patients. CONCLUSION: Although MLL gene rearrangements are generally associated with a dismal outcome in ALL, two distinct subsets with MLL-ENL fusions have an excellent prognosis. Our results suggest that patients with this genetic abnormality who have T-cell ALL or are 1 to 9 years of age should not be considered candidates for hematopoietic stem-cell transplantation during their first remission.
Authors: Juliann Chmielecki; Mark Bailey; Jie He; Julia Elvin; Jo-Anne Vergilio; Shakti Ramkissoon; James Suh; Garrett M Frampton; James X Sun; Samantha Morley; Daniel Spritz; Siraj Ali; Laurie Gay; Rachel L Erlich; Jeffrey S Ross; Joana Buxhaku; Hilary Davies; Vinny Faso; Alexis Germain; Blair Glanville; Vincent A Miller; Philip J Stephens; Katherine A Janeway; John M Maris; Soheil Meshinchi; Trevor J Pugh; Jack F Shern; Doron Lipson Journal: Cancer Res Date: 2017-01-09 Impact factor: 12.701
Authors: Joanne M Hilden; Patricia A Dinndorf; Sharon O Meerbaum; Harland Sather; Doojduen Villaluna; Nyla A Heerema; Ron McGlennen; Franklin O Smith; William G Woods; Wanda L Salzer; Helen S Johnstone; Zoann Dreyer; Gregory H Reaman Journal: Blood Date: 2006-03-23 Impact factor: 22.113
Authors: Chester K Yarbrough; S Kathleen Bandt; Kyle Hurth; Jennifer A Wambach; Rakesh Rao; Shashikant Kulkarni; Francis V White; John L Frater; Jeffrey R Leonard Journal: Cureus Date: 2015-07-27