title: NUP214-ABL1 in adult T-ALL: the GMALL study group experience.
abstract: The NUP214-ABL1 fusion gene in T-cell acute lymphoblastic leukemia (T-ALL) has recently been identified as a possible target for imatinib and related tyrosine kinase inhibitors, but exact data regarding the prognostic impact and frequency of the several putative NUP214-ABL1 mRNA transcripts are still missing. We investigated 279 adult patients with T-ALL treated within the framework of the GMALL 5/93 and 6/99 therapy trials for NUP214-ABL1 by using a novel multiplex real-time, quantitative polymerase chain reaction (PCR). Eleven (3.9%) patients were NUP214-ABL1 positive, and 5 different transcripts were observed; 8 patients had a thymic immunophenotype, 1 had an early T-cell immunophenotype, and 2 had a mature T-cell immunophenotype. NUP214-ABL1-positive and -negative patients did not differ significantly in their major clinical features. In contrast to previous reports suggesting an adverse clinical course for NUP214-ABL1-positive patients, no significant difference in overall survival was observed. Based on the results, we have established and tested a novel PCR method for simplified detection of the NUP214-ABL1 fusion gene.
authors.affiliation: Charité Universitätsmedizin Berlin, Campus Benjamin Franklin, Medizinische Klinik III, Hindenburgdamm 30, 12200 Berlin, Germany. thomas.burmeister@charite.de.
authors.forename: Thomas
authors.initials: T
authors.lastname: Burmeister
authors.forename: Nicola
authors.initials: N
authors.lastname: Gökbuget
authors.forename: Richard
authors.initials: R
authors.lastname: Reinhardt
authors.forename: Harald
authors.initials: H
authors.lastname: Rieder
authors.forename: Dieter
authors.initials: D
authors.lastname: Hoelzer
authors.forename: Stefan
authors.initials: S
authors.lastname: Schwartz
mesh_terms: D000293:Adolescent; D000328:Adult; D001549:Benzamides; D005260:Female; D006801:Humans; D000068877:Imatinib Mesylate; D016130:Immunophenotyping; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000235:genetics* / Q000401:mortality / Q000473:pathology; D008297:Male; D028861:Nuclear Pore Complex Proteins / Q000032:analysis / Q000235:genetics*; D015514:Oncogene Proteins, Fusion / Q000032:analysis / Q000235:genetics*; D010879:Piperazines / Q000627:therapeutic use; D016133:Polymerase Chain Reaction; D011379:Prognosis; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors; D016315:Proto-Oncogene Proteins c-abl / Q000032:analysis / Q000235:genetics*; D011743:Pyrimidines / Q000627:therapeutic use; D012333:RNA, Messenger / Q000032:analysis; D015996:Survival Rate
chemical_list: D001549:Benzamides; C085914:NUP214 protein, human; D028861:Nuclear Pore Complex Proteins; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D011743:Pyrimidines; D012333:RNA, Messenger; D000068877:Imatinib Mesylate; D011505:Protein-Tyrosine Kinases; D016315:Proto-Oncogene Proteins c-abl