NUP214-ABL1 in adult T-ALL: the GMALL study group experience

Blood. 2006 Nov 15;108(10):3556-9. doi: 10.1182/blood-2006-04-014514. Epub 2006 Jul 27.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Benzamides
  • Female
  • Humans
  • Imatinib Mesylate
  • Immunophenotyping
  • Leukemia-Lymphoma, Adult T-Cell / genetics*
  • Leukemia-Lymphoma, Adult T-Cell / mortality
  • Leukemia-Lymphoma, Adult T-Cell / pathology
  • Male
  • Nuclear Pore Complex Proteins / analysis
  • Nuclear Pore Complex Proteins / genetics*
  • Oncogene Proteins, Fusion / analysis
  • Oncogene Proteins, Fusion / genetics*
  • Piperazines / therapeutic use
  • Polymerase Chain Reaction
  • Prognosis
  • Protein-Tyrosine Kinases / antagonists & inhibitors
  • Proto-Oncogene Proteins c-abl / analysis
  • Proto-Oncogene Proteins c-abl / genetics*
  • Pyrimidines / therapeutic use
  • RNA, Messenger / analysis
  • Survival Rate

Substances

  • Benzamides
  • NUP214 protein, human
  • Nuclear Pore Complex Proteins
  • Oncogene Proteins, Fusion
  • Piperazines
  • Pyrimidines
  • RNA, Messenger
  • Imatinib Mesylate
  • Protein-Tyrosine Kinases
  • Proto-Oncogene Proteins c-abl