title: Advanced phase chronic myeloid leukaemia (CML) in the tyrosine kinase inhibitor era - a report from the Swedish CML register.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Medical Sciences, Uppsala University, Uppsala, Sweden.|Section of Haematology, Uppsala University Hospital, Uppsala, Sweden.
authors.forename: Stina
authors.initials: S
authors.lastname: Söderlund
authors.affiliation: Department of Haematology, Karolinska University Hospital, Stockholm, Sweden.|Department of Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Torsten
authors.initials: T
authors.lastname: Dahlén
authors.affiliation: Regional Cancer Centre Uppsala-Örebro, Uppsala, Sweden.
authors.forename: Fredrik
authors.initials: F
authors.lastname: Sandin
authors.affiliation: Department of Medical Sciences, Uppsala University, Uppsala, Sweden.|Section of Haematology, Uppsala University Hospital, Uppsala, Sweden.
authors.forename: Ulla
authors.initials: U
authors.lastname: Olsson-Strömberg
authors.affiliation: Department of Haematology, Linköping University Hospital, Linköping, Sweden.
authors.forename: Maria
authors.initials: M
authors.lastname: Creignou
authors.affiliation: Department of Haematology, Linköping University Hospital, Linköping, Sweden.
authors.forename: Arta
authors.initials: A
authors.lastname: Dreimane
authors.affiliation: Department of Haematology, Oncology and Radiation Physics, Skåne University Hospital, Lund, Sweden.
authors.forename: Anna
authors.initials: A
authors.lastname: Lübking
authors.affiliation: Department of Haematology, Umeå University Hospital, Umeå, Sweden.
authors.forename: Berit
authors.initials: B
authors.lastname: Markevärn
authors.affiliation: Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden.
authors.forename: Anders
authors.initials: A
authors.lastname: Själander
authors.affiliation: Section of Haematology and Coagulation, Sahlgrenska University Hospital, Göteborg, Sweden.
authors.forename: Hans
authors.initials: H
authors.lastname: Wadenvik
authors.affiliation: Department of Haematology, Karolinska University Hospital, Stockholm, Sweden.|Department of Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Leif
authors.initials: L
authors.lastname: Stenke
authors.affiliation: Department of Haematology, Oncology and Radiation Physics, Skåne University Hospital, Lund, Sweden.
authors.forename: Johan
authors.initials: J
authors.lastname: Richter
authors.affiliation: Department of Medical Sciences, Uppsala University, Uppsala, Sweden.|Section of Haematology, Uppsala University Hospital, Uppsala, Sweden.
authors.forename: Martin
authors.initials: M
authors.lastname: Höglund
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001752:Blast Crisis; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D015466:Leukemia, Myeloid, Chronic-Phase / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011159:Population Surveillance; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D012042:Registries; D013548:Sweden / Q000453:epidemiology; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents; D047428:Protein Kinase Inhibitors
keywords: accelerated phase; blast crisis; chronic myeloid leukaemia; population-based; tyrosine kinase inhibitor