title: The therapeutic response and clinical outcome of adults with ALL1(MLL)/AF4 fusion positive acute lymphoblastic leukemia according to the GIMEMA experience.
abstract: The clinical outcome of 21 adults with ALL1(MLL)/AF4 positive acute lymphoblastic leukemia enrolled in the GIMEMA LAL 2000 trial and of 25 patients entered into the previous 0496 study is reported. LAL 2000 included more intensive consolidation and transplants. Complete remission rates were 90% and 88% in the LAL 2000 and 0496 trials, respectively. Fifteen patients were transplanted (5 autologous, 10 allogeneic). At 36 months, overall and disease free survivals were 32.9%, 31.8%, 28% and 27.3%, in LAL 2000 and 0496 trials, respectively. Relapses remained the main reason of failure occurring in 10 and 16 of the 19 and 22 responding patients. In the LAL 2000 study, 4 relapses were observed before transplant. Thus, ALL1(MLL)/AF4 abnormality characterized a subset of patients with adverse prognosis in which the overall strategy adopted in the LAL 2000 study, rather than transplants per se, failed to improve the patient clinical outcome.
authors.affiliation: Hematology, University "Sapienza", Via Benevento 6, 00161 Rome; Italy. cimino@bce.uniroma1.it
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Cimino
authors.forename: Natalia
authors.initials: N
authors.lastname: Cenfra
authors.forename: Loredana
authors.initials: L
authors.lastname: Elia
authors.forename: Simona
authors.initials: S
authors.lastname: Sica
authors.forename: Mario
authors.initials: M
authors.lastname: Luppi
authors.forename: Giovanna
authors.initials: G
authors.lastname: Meloni
authors.forename: Marco
authors.initials: M
authors.lastname: Vignetti
authors.forename: Francesca
authors.initials: F
authors.lastname: Paoloni
authors.forename: Robin
authors.initials: R
authors.lastname: Foà
authors.forename: Franco
authors.initials: F
authors.lastname: Mandelli
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D014408:Biomarkers, Tumor / Q000235:genetics*; D015331:Cohort Studies; D004268:DNA-Binding Proteins / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation; D011495:Histone-Lysine N-Methyltransferase; D006801:Humans; D008297:Male; D008875:Middle Aged; D051788:Myeloid-Lymphoid Leukemia Protein / Q000235:genetics*; D009687:Nuclear Proteins / Q000235:genetics*; D015514:Oncogene Proteins, Fusion; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics* / Q000628:therapy*; D012074:Remission Induction; D035602:Transcriptional Elongation Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; D004268:DNA-Binding Proteins; C497422:KMT2A protein, human; D009687:Nuclear Proteins; D015514:Oncogene Proteins, Fusion; D035602:Transcriptional Elongation Factors; D051788:Myeloid-Lymphoid Leukemia Protein; C081449:AFF1 protein, human; D011495:Histone-Lysine N-Methyltransferase