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Clinical and cytogenetic features of a population-based consecutive series of 285 pediatric T-cell acute lymphoblastic leukemias: Rare T-cell receptor gene rearrangements are associated with poor outcome.

In English

Författare

  • Kristina Karrman
  • Erik Forestier
  • Mats Heyman
  • Mette K Andersen
  • Kirsi Autio
  • Elisabeth Blennow
  • Georg Borgström
  • Hans Ehrencrona
  • Irina Golovleva
  • Sverre Heim
  • Kristiina Heinonen
  • Randi Hovland
  • Johann H Johannsson
  • Gitte Kerndrup
  • Ann Nordgren
  • Lars Palmqvist
  • Bertil Johansson
Visa allt

Summary, in English

Clinical characteristics and cytogenetic aberrations were ascertained and reviewed in a population-based consecutive series of 285 pediatric T-cell acute lymphoblastic leukemias (T-ALLs) diagnosed between 1992 and 2006 in the Nordic countries. Informative karyotypic results were obtained in 249 (87%) cases, of which 119 (48%) were cytogenetically abnormal. Most (62%) of the aberrant T-ALLs were pseudodiploid. Structural changes were more common than numerical ones; 86% displayed at least one structural abnormality and 41% at least one numerical anomaly. The most frequent abnormalities were T-cell receptor (TCR) gene rearrangements (20%) [TCR;11p13 (10%), TCR;10q24 (3%), TCR;other (8%)], del(9p) (17%), +8 (14%), del(6q) (12%), and 11q23 rearrangements (6%). The TCR;other group comprised the rare rearrangements t(X;14)(p11;q11), t(X;7)(q22;q34), t(1;14)(p32;q11), ins(14;5)(q11;q?q?), inv(7)(p15q34), t(8;14)(q24;q11), t(7;11)(q34;p15), and t(12;14)(p13;q11). The clinical characteristics of this Nordic patient cohort agreed well with previous larger series, with a median age of 9.0 years, male predominance (male/female ratio 3.1), median white blood cell (WBC) count of 66.5 x 10(9)/l, and a high incidence of mediastinal mass and central nervous system involvement (59% and 9.5%, respectively). These features did not differ significantly among the various genetic subgroups. 5-year event-free survival (EFS) and overall survival for all patients were 0.61 (+/-0.03) and 0.67 (+/-0.03), respectively. In a multivariate analysis, two factors affected negatively the EFS, namely a WBC count of > or =200 x 10(9)/l (P < 0.001) and the presence of rare TCR rearrangements (P = 0.001). In conclusion, in this large series of childhood T-ALLs from the Nordic countries, the cytogenetic findings were not associated with risk of therapy failure with the exception of the TCR;other group. However, further prospective and collaborative investigations of this genetically heterogeneous entity are needed to confirm these results.

Publiceringsår

2009

Språk

Engelska

Sidor

795-805

Publikation/Tidskrift/Serie

Genes, Chromosomes and Cancer

Volym

48

Issue

9

Dokumenttyp

Artikel i tidskrift

Förlag

John Wiley & Sons Inc.

Ämne

  • Medical Genetics

Nyckelord

  • Multivariate Analysis
  • Male
  • Kaplan-Meier Estimate
  • Humans
  • T-Lymphocyte
  • Gene Rearrangement
  • Female
  • Cytogenetic Analysis
  • Cohort Studies
  • Chromosome Aberrations
  • Preschool
  • Child
  • Adolescent
  • Chi-Square Distribution
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma
  • Prognosis
  • Proportional Hazards Models
  • Receptors
  • Antigen
  • T-Cell

Status

Published

ISBN/ISSN/Övrigt

  • ISSN: 1045-2257