Premium
Acute promyelocytic leukaemia is characterized by stable incidence and improved survival that is restricted to patients managed in leukaemia referral centres: a pan‐ C anadian epidemiological study
Author(s) -
Paulson Kristjan,
Serebrin Anna,
Lambert Pascal,
Bergeron Julie,
Everett Janeve,
Kew Andrea,
Jones David,
Mahmud Salah,
Meloche Catherine,
Sabloff Mitchell,
Sharif Ismail,
Storring John,
Turner Donna,
Seftel Matthew D.
Publication year - 2014
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.12931
Subject(s) - medicine , referral , incidence (geometry) , epidemiology , pediatrics , cohort , cancer registry , acute promyelocytic leukemia , cohort study , malignancy , family medicine , retinoic acid , biochemistry , physics , chemistry , optics , gene
Summary Timely diagnosis and care are major determinants of the outcome in acute promyelocytic leukaemia ( APL ), a malignancy whose incidence may be increasing. The C anadian C ancer R egistry ( CCR ) and health system represent valuable settings to study APL epidemiology. We analysed the CCR , which contains data on all C anadians with APL . To provide clinical information lacking in the CCR , we obtained data from five leukaemia referral centres during a similar time period. Between 1993 and 2007, there were 399 APL in C anada. Age‐standardized incidence was 0·083/100 000 and was stable over time. The early death ( ED ) rate was 21·8% (10·6% in patients <50 years old and 35·5% for those aged >50 years), with no improvement over time. Five‐year overall survival ( OS ) was 54·6% (73·3% in patients <50 years; 29·1% older patients). In the referral cohort, 131 patients were diagnosed between 1999 and 2010. ED was 14·6% and 2‐year OS was 76·5%. Within this cohort, ED and OS improved over time, although advanced patient age remained an adverse determinant of OS . In Canada, APL incidence is unexpectedly low and temporally stable. ED was higher than reported in clinical trials, but similar to reports from other registries. In contrast, ED was lower in referral centres and improved with time.