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Additional cytogenetic abnormalities and variant t(9;22) at the diagnosis of childhood chronic myeloid leukemia: The experience of the International Registry for Chronic Myeloid Leukemia in Children and Adolescents

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  • Frédéric Millot
  • Christelle Dupraz
  • Joelle Guilhot
  • Meinolf Suttorp
  • Françoise Brizard
  • Thierry Leblanc
  • Adalet Meral Güneş
  • Petr Sedlacek
  • Evelyne De Bont
  • Chi Kong Li
  • Krzysztof Kalwak
  • Birgitte Lausen
  • Srdjana Culic
  • Michael Dworzak
  • Emilia Kaiserova
  • Barbara De Moerloose
  • Farah Roula
  • Andrea Biondi
  • André Baruchel
  • François Guilhot
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BACKGROUND: In the adult population with newly diagnosed chronic myeloid leukemia (CML), variant translocations are usually not considered to be impairing the prognosis, whereas some additional cytogenetic abnormalities (ACAs) are associated with a negative impact on survival. Because of the rarity of CML in the pediatric population, such abnormalities have not been investigated in a large group of children with CML.

METHODS: The prognostic relevance of variant t(9;22) and ACAs at diagnosis was assessed in 301 children with CML in the chronic phase who were enrolled in the International Registry for Chronic Myeloid Leukemia in Children and Adolescents.

RESULTS: Overall, 19 children (6.3%) presented with additional cytogenetic findings at diagnosis: 5 children (1.7%) had a variant t(9;22) translocation, 13 children (4.3%) had ACAs, and 1 had both. At 3 years, for children with a classic translocation, children with ACAs, and children with a variant t(9;22) translocation who were treated with imatinib as frontline therapy, the probability of progression-free survival (PFS) was 95% (95% confidence interval [CI], 91%-97%), 100%, and 75% (95% CI, 13%-96%), respectively, and the probability of overall survival (OS) was 98% (95% CI, 95%-100%), 100% (95% CI, 43%-98%), and 75% (95% CI, 13%-96%), respectively. No statistical difference was observed between the patients with classic cytogenetic findings and those with additional chromosomal abnormalities in terms of PFS and OS.

CONCLUSIONS: In contrast to adults with CML, additional chromosomal abnormalities observed at diagnosis do not seem to have a significant prognostic impact. Cancer 2017;123:3609-16. © 2017 American Cancer Society.

OriginalsprogEngelsk
TidsskriftCancer
Vol/bind123
Udgave nummer18
Sider (fra-til)3609-3616
Antal sider8
ISSN0008-543X
DOI
StatusUdgivet - 15 sep. 2017

ID: 52166751