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Outcome of ETV6/RUNX1-positive childhood acute lymphoblastic leukaemia in the NOPHO-ALL-1992 protocol: frequent late relapses but good overall survival

Forestier, Erik; Heyman, Mats; Andersen, Mette K.; Autio, Kirsi; Blennow, Elisabeth; Borgstrom, Georg; Golovleva, Irina; Heim, Sverre; Heinonen, Kristina and Hovland, Randi, et al. (2008) In British Journal of Haematology 140(6). p.665-672
Abstract
The prognostic impact of t(12;21)(p13;q22) [ETV6/RUNX1 fusion] in paediatric acute lymphoblastic leukaemia (ALL) has been extensively debated, particularly with regard to the frequency of late relapses and appropriate treatment regimens. We have retrospectively collected 679 ALLs with known ETV6/RUNX1 status, as ascertained by fluorescence in situ hybridization or reverse-transcription polymerase chain reaction, treated according to the Nordic Society of Paediatric Haematology and Oncology -ALL-1992 protocol. The assigned risk groups/treatment modalities for the 171 (25%) patients with t(12;21)-positive ALLs were 74 (43%) standard risk, 71 (42%) intermediate risk and 26 (15%) high risk. The 5- and 10-year event-free survival (EFS) of the... (More)
The prognostic impact of t(12;21)(p13;q22) [ETV6/RUNX1 fusion] in paediatric acute lymphoblastic leukaemia (ALL) has been extensively debated, particularly with regard to the frequency of late relapses and appropriate treatment regimens. We have retrospectively collected 679 ALLs with known ETV6/RUNX1 status, as ascertained by fluorescence in situ hybridization or reverse-transcription polymerase chain reaction, treated according to the Nordic Society of Paediatric Haematology and Oncology -ALL-1992 protocol. The assigned risk groups/treatment modalities for the 171 (25%) patients with t(12;21)-positive ALLs were 74 (43%) standard risk, 71 (42%) intermediate risk and 26 (15%) high risk. The 5- and 10-year event-free survival (EFS) of the 171 patients was 80% and 75% respectively, with no significant differences among the three risk groups. Most of the relapses occurred in boys and were late, with almost 50% of all relapses occurring >= 5 years after diagnosis. Of all relapses after 6 years, 80% occurred in the t(12;21)-positive group. The overall survival was 94% at 5 years and 88% at 10 years; thus, the treatment of patients in second or later remission is usually successful. As yet, there is no reliable plateau in the EFS curve, a fact that raises the question as to when the prognostic ramifications of ALLs harbouring ETV6/RUNX1 should be evaluated. (Less)
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publication status
published
subject
keywords
childhood, prognosis, relapse, acute lymphoblastic leukaemia, q22), 21)(p13, ETV6/RUNX1, t(12
in
British Journal of Haematology
volume
140
issue
6
pages
665 - 672
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • wos:000253505100007
  • scopus:39749177335
ISSN
0007-1048
DOI
10.1111/j.1365-2141.2008.06980.x
language
English
LU publication?
yes
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9133f26e-4cd2-4a9c-8dd7-e2f124cf9ee5 (old id 1193852)
date added to LUP
2008-09-10 09:39:43
date last changed
2017-10-22 03:37:57
@article{9133f26e-4cd2-4a9c-8dd7-e2f124cf9ee5,
  abstract     = {The prognostic impact of t(12;21)(p13;q22) [ETV6/RUNX1 fusion] in paediatric acute lymphoblastic leukaemia (ALL) has been extensively debated, particularly with regard to the frequency of late relapses and appropriate treatment regimens. We have retrospectively collected 679 ALLs with known ETV6/RUNX1 status, as ascertained by fluorescence in situ hybridization or reverse-transcription polymerase chain reaction, treated according to the Nordic Society of Paediatric Haematology and Oncology -ALL-1992 protocol. The assigned risk groups/treatment modalities for the 171 (25%) patients with t(12;21)-positive ALLs were 74 (43%) standard risk, 71 (42%) intermediate risk and 26 (15%) high risk. The 5- and 10-year event-free survival (EFS) of the 171 patients was 80% and 75% respectively, with no significant differences among the three risk groups. Most of the relapses occurred in boys and were late, with almost 50% of all relapses occurring >= 5 years after diagnosis. Of all relapses after 6 years, 80% occurred in the t(12;21)-positive group. The overall survival was 94% at 5 years and 88% at 10 years; thus, the treatment of patients in second or later remission is usually successful. As yet, there is no reliable plateau in the EFS curve, a fact that raises the question as to when the prognostic ramifications of ALLs harbouring ETV6/RUNX1 should be evaluated.},
  author       = {Forestier, Erik and Heyman, Mats and Andersen, Mette K. and Autio, Kirsi and Blennow, Elisabeth and Borgstrom, Georg and Golovleva, Irina and Heim, Sverre and Heinonen, Kristina and Hovland, Randi and Johannsson, Johann H. and Kerndrup, Gitte and Nordgren, Ann and Rosenquist, Richard and Swolin, Birgitta and Johansson, Bertil},
  issn         = {0007-1048},
  keyword      = {childhood,prognosis,relapse,acute lymphoblastic leukaemia,q22),21)(p13,ETV6/RUNX1,t(12},
  language     = {eng},
  number       = {6},
  pages        = {665--672},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {British Journal of Haematology},
  title        = {Outcome of ETV6/RUNX1-positive childhood acute lymphoblastic leukaemia in the NOPHO-ALL-1992 protocol: frequent late relapses but good overall survival},
  url          = {http://dx.doi.org/10.1111/j.1365-2141.2008.06980.x},
  volume       = {140},
  year         = {2008},
}