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Advanced phase chronic myeloid leukaemia (CML) in the tyrosine kinase inhibitor era - a report from the Swedish CML register

Söderlund, Stina; Dahlén, Torsten; Sandin, Fredrik; Olsson-Strömberg, Ulla; Creignou, Maria; Dreimane, Arta; Lübking, Anna LU ; Markevärn, Berit; Själander, Anders and Wadenvik, Hans, et al. (2017) In European Journal of Haematology1987-01-01+01:00 98(1). p.57-66
Abstract

Objectives: The primary goal in management of chronic phase (CP) chronic myeloid leukaemia (CML) is to prevent disease progression to accelerated phase (AP) or blast crisis (BC). We have evaluated progression rates in a decentralised healthcare setting and characterised patients progressing to AP/BC on TKI treatment. Methods: Using data from the Swedish CML register, we identified CP-CML patients diagnosed 2007-2011 who progressed to AP/BC within 2 yrs from diagnosis (n = 18) as well as patients diagnosed in advanced phase during 2007-2012 (n = 36) from a total of 544 newly diagnosed CML cases. We evaluated baseline characteristics, progression rates, outcome and adherence to guidelines for monitoring and treatment. Results: The... (More)

Objectives: The primary goal in management of chronic phase (CP) chronic myeloid leukaemia (CML) is to prevent disease progression to accelerated phase (AP) or blast crisis (BC). We have evaluated progression rates in a decentralised healthcare setting and characterised patients progressing to AP/BC on TKI treatment. Methods: Using data from the Swedish CML register, we identified CP-CML patients diagnosed 2007-2011 who progressed to AP/BC within 2 yrs from diagnosis (n = 18) as well as patients diagnosed in advanced phase during 2007-2012 (n = 36) from a total of 544 newly diagnosed CML cases. We evaluated baseline characteristics, progression rates, outcome and adherence to guidelines for monitoring and treatment. Results: The cumulative progression rate at 2 yrs was 4.3%. All 18 progression cases had been treated with imatinib, and six progressed within 6 months. High-risk EUTOS score was associated to a higher risk of progression. Insufficient cytogenetic and/or molecular monitoring was found in 33%. Median survival after transformation during TKI treatment was 1.4 yrs. In those presenting with BC and AP, median survival was 1.6 yrs and not reached, respectively. Conclusion: In this population-based setting, progression rates appear comparable to that reported from clinical trials, with similar dismal patient outcome. Improved adherence to CML guidelines may minimise the risk of disease progression.

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published
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keywords
Accelerated phase, Blast crisis, Chronic myeloid leukaemia, Population-based, Tyrosine kinase inhibitor
in
European Journal of Haematology1987-01-01+01:00
volume
98
issue
1
pages
57 - 66
publisher
Wiley-Blackwell
external identifiers
  • scopus:84994631357
  • wos:000393166600009
ISSN
0902-4441
DOI
10.1111/ejh.12785
language
English
LU publication?
yes
id
a468f62e-cb25-48b1-a61e-5c13221c3bb3
date added to LUP
2016-12-05 10:45:29
date last changed
2018-04-08 04:51:30
@article{a468f62e-cb25-48b1-a61e-5c13221c3bb3,
  abstract     = {<p>Objectives: The primary goal in management of chronic phase (CP) chronic myeloid leukaemia (CML) is to prevent disease progression to accelerated phase (AP) or blast crisis (BC). We have evaluated progression rates in a decentralised healthcare setting and characterised patients progressing to AP/BC on TKI treatment. Methods: Using data from the Swedish CML register, we identified CP-CML patients diagnosed 2007-2011 who progressed to AP/BC within 2 yrs from diagnosis (n = 18) as well as patients diagnosed in advanced phase during 2007-2012 (n = 36) from a total of 544 newly diagnosed CML cases. We evaluated baseline characteristics, progression rates, outcome and adherence to guidelines for monitoring and treatment. Results: The cumulative progression rate at 2 yrs was 4.3%. All 18 progression cases had been treated with imatinib, and six progressed within 6 months. High-risk EUTOS score was associated to a higher risk of progression. Insufficient cytogenetic and/or molecular monitoring was found in 33%. Median survival after transformation during TKI treatment was 1.4 yrs. In those presenting with BC and AP, median survival was 1.6 yrs and not reached, respectively. Conclusion: In this population-based setting, progression rates appear comparable to that reported from clinical trials, with similar dismal patient outcome. Improved adherence to CML guidelines may minimise the risk of disease progression.</p>},
  author       = {Söderlund, Stina and Dahlén, Torsten and Sandin, Fredrik and Olsson-Strömberg, Ulla and Creignou, Maria and Dreimane, Arta and Lübking, Anna and Markevärn, Berit and Själander, Anders and Wadenvik, Hans and Stenke, Leif and Richter, Johan and Höglund, Martin},
  issn         = {0902-4441},
  keyword      = {Accelerated phase,Blast crisis,Chronic myeloid leukaemia,Population-based,Tyrosine kinase inhibitor},
  language     = {eng},
  number       = {1},
  pages        = {57--66},
  publisher    = {Wiley-Blackwell},
  series       = {European Journal of Haematology1987-01-01+01:00},
  title        = {Advanced phase chronic myeloid leukaemia (CML) in the tyrosine kinase inhibitor era - a report from the Swedish CML register},
  url          = {http://dx.doi.org/10.1111/ejh.12785},
  volume       = {98},
  year         = {2017},
}