title: Risk and Cause of Death in Patients Diagnosed With Myeloproliferative Neoplasms in Sweden Between 1973 and 2005: A Population-Based Study.
abstract: OBJECTIVE
  Myeloproliferative neoplasms (MPNs) are associated with a shortened life expectancy. We assessed causes of death in patients with MPN and matched controls using both relative risks and absolute probabilities in the presence of competing risks.
  
  
  METHODS
  From Swedish registries, we identified 9,285 patients with MPN and 35,769 matched controls. A flexible parametric model was used to estimate cause-specific hazard ratios (HRs) of death and cumulative incidence functions, each with 95% CIs.
  
  
  RESULTS
  In patients with MPN, the HRs of death from hematologic malignancies and infections were 92.8 (95% CI, 70.0 to 123.1) and 2.7 (95% CI, 2.4 to 3.1), respectively. In patients age 70 to 79 years at diagnosis (the largest patient group), the HRs of death from cardiovascular and cerebrovascular disease were 1.5 (95% CI, 1.4 to 1.7) and 1.5 (95% CI, 1.3 to 1.8), respectively; all were statistically significantly elevated compared with those of controls. In the same age group, no difference was observed in the 10-year probability of death resulting from cardiovascular disease in patients with MPN versus controls (16.8% v 15.2%) or cerebrovascular disease (5.6% v 5.2%). In patients age 50 to 59 years at diagnosis, the 10-year probability of death resulting from cardiovascular and cerebrovascular disease was elevated, 4.2% versus 2.1% and 1.9% versus 0.4%, respectively. Survival in patients with MPN increased over time, mainly because of decreased probabilities of dying as a result of hematologic malignancies, infections, and, in young patients, cardiovascular disease.
  
  
  CONCLUSIONS
  Patients with MPN had an overall higher mortality rate than that of matched controls, primarily because of hematologic malignancy, infections, and vascular events in younger patients. Evidently, there is still a need for effective disease-modifying agents to improve patient outcomes.
authors.affiliation: Malin Hultcrantz, Sigurdur Y. Kristinsson, Åsa R. Derolf, and Magnus Björkholm, Karolinska University Hospital; Malin Hultcrantz, Sigurdur Y. Kristinsson, Therese M.-L. Andersson, Åsa R. Derolf, Sandra Eloranta, Paul W. Dickman, Paul C. Lambert, and Magnus Björkholm, Karolinska Institutet; Jan Samuelsson, South Hospital, Stockholm, Sweden; Sally R. Wilkes, University of Nottingham, Nottingham; Paul C. Lambert, University of Leicester, Leicester, United Kingdom; Sigurdur Y. Kristinsson, University of Iceland and Landspitali National University Hospital, Reykjavik, Iceland; and Ola Landgren, Memorial Sloan Kettering Cancer Center, New York, NY. malin.hultcrantz@ki.se.
authors.forename: Malin
authors.initials: M
authors.lastname: Hultcrantz
authors.affiliation: Malin Hultcrantz, Sigurdur Y. Kristinsson, Åsa R. Derolf, and Magnus Björkholm, Karolinska University Hospital; Malin Hultcrantz, Sigurdur Y. Kristinsson, Therese M.-L. Andersson, Åsa R. Derolf, Sandra Eloranta, Paul W. Dickman, Paul C. Lambert, and Magnus Björkholm, Karolinska Institutet; Jan Samuelsson, South Hospital, Stockholm, Sweden; Sally R. Wilkes, University of Nottingham, Nottingham; Paul C. Lambert, University of Leicester, Leicester, United Kingdom; Sigurdur Y. Kristinsson, University of Iceland and Landspitali National University Hospital, Reykjavik, Iceland; and Ola Landgren, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Sally R
authors.initials: SR
authors.lastname: Wilkes
authors.affiliation: Malin Hultcrantz, Sigurdur Y. Kristinsson, Åsa R. Derolf, and Magnus Björkholm, Karolinska University Hospital; Malin Hultcrantz, Sigurdur Y. Kristinsson, Therese M.-L. Andersson, Åsa R. Derolf, Sandra Eloranta, Paul W. Dickman, Paul C. Lambert, and Magnus Björkholm, Karolinska Institutet; Jan Samuelsson, South Hospital, Stockholm, Sweden; Sally R. Wilkes, University of Nottingham, Nottingham; Paul C. Lambert, University of Leicester, Leicester, United Kingdom; Sigurdur Y. Kristinsson, University of Iceland and Landspitali National University Hospital, Reykjavik, Iceland; and Ola Landgren, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Sigurdur Y
authors.initials: SY
authors.lastname: Kristinsson
authors.affiliation: Malin Hultcrantz, Sigurdur Y. Kristinsson, Åsa R. Derolf, and Magnus Björkholm, Karolinska University Hospital; Malin Hultcrantz, Sigurdur Y. Kristinsson, Therese M.-L. Andersson, Åsa R. Derolf, Sandra Eloranta, Paul W. Dickman, Paul C. Lambert, and Magnus Björkholm, Karolinska Institutet; Jan Samuelsson, South Hospital, Stockholm, Sweden; Sally R. Wilkes, University of Nottingham, Nottingham; Paul C. Lambert, University of Leicester, Leicester, United Kingdom; Sigurdur Y. Kristinsson, University of Iceland and Landspitali National University Hospital, Reykjavik, Iceland; and Ola Landgren, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Therese M-L
authors.initials: TM
authors.lastname: Andersson
authors.affiliation: Malin Hultcrantz, Sigurdur Y. Kristinsson, Åsa R. Derolf, and Magnus Björkholm, Karolinska University Hospital; Malin Hultcrantz, Sigurdur Y. Kristinsson, Therese M.-L. Andersson, Åsa R. Derolf, Sandra Eloranta, Paul W. Dickman, Paul C. Lambert, and Magnus Björkholm, Karolinska Institutet; Jan Samuelsson, South Hospital, Stockholm, Sweden; Sally R. Wilkes, University of Nottingham, Nottingham; Paul C. Lambert, University of Leicester, Leicester, United Kingdom; Sigurdur Y. Kristinsson, University of Iceland and Landspitali National University Hospital, Reykjavik, Iceland; and Ola Landgren, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Åsa R
authors.initials: ÅR
authors.lastname: Derolf
authors.affiliation: Malin Hultcrantz, Sigurdur Y. Kristinsson, Åsa R. Derolf, and Magnus Björkholm, Karolinska University Hospital; Malin Hultcrantz, Sigurdur Y. Kristinsson, Therese M.-L. Andersson, Åsa R. Derolf, Sandra Eloranta, Paul W. Dickman, Paul C. Lambert, and Magnus Björkholm, Karolinska Institutet; Jan Samuelsson, South Hospital, Stockholm, Sweden; Sally R. Wilkes, University of Nottingham, Nottingham; Paul C. Lambert, University of Leicester, Leicester, United Kingdom; Sigurdur Y. Kristinsson, University of Iceland and Landspitali National University Hospital, Reykjavik, Iceland; and Ola Landgren, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Sandra
authors.initials: S
authors.lastname: Eloranta
authors.affiliation: Malin Hultcrantz, Sigurdur Y. Kristinsson, Åsa R. Derolf, and Magnus Björkholm, Karolinska University Hospital; Malin Hultcrantz, Sigurdur Y. Kristinsson, Therese M.-L. Andersson, Åsa R. Derolf, Sandra Eloranta, Paul W. Dickman, Paul C. Lambert, and Magnus Björkholm, Karolinska Institutet; Jan Samuelsson, South Hospital, Stockholm, Sweden; Sally R. Wilkes, University of Nottingham, Nottingham; Paul C. Lambert, University of Leicester, Leicester, United Kingdom; Sigurdur Y. Kristinsson, University of Iceland and Landspitali National University Hospital, Reykjavik, Iceland; and Ola Landgren, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Jan
authors.initials: J
authors.lastname: Samuelsson
authors.affiliation: Malin Hultcrantz, Sigurdur Y. Kristinsson, Åsa R. Derolf, and Magnus Björkholm, Karolinska University Hospital; Malin Hultcrantz, Sigurdur Y. Kristinsson, Therese M.-L. Andersson, Åsa R. Derolf, Sandra Eloranta, Paul W. Dickman, Paul C. Lambert, and Magnus Björkholm, Karolinska Institutet; Jan Samuelsson, South Hospital, Stockholm, Sweden; Sally R. Wilkes, University of Nottingham, Nottingham; Paul C. Lambert, University of Leicester, Leicester, United Kingdom; Sigurdur Y. Kristinsson, University of Iceland and Landspitali National University Hospital, Reykjavik, Iceland; and Ola Landgren, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Ola
authors.initials: O
authors.lastname: Landgren
authors.affiliation: Malin Hultcrantz, Sigurdur Y. Kristinsson, Åsa R. Derolf, and Magnus Björkholm, Karolinska University Hospital; Malin Hultcrantz, Sigurdur Y. Kristinsson, Therese M.-L. Andersson, Åsa R. Derolf, Sandra Eloranta, Paul W. Dickman, Paul C. Lambert, and Magnus Björkholm, Karolinska Institutet; Jan Samuelsson, South Hospital, Stockholm, Sweden; Sally R. Wilkes, University of Nottingham, Nottingham; Paul C. Lambert, University of Leicester, Leicester, United Kingdom; Sigurdur Y. Kristinsson, University of Iceland and Landspitali National University Hospital, Reykjavik, Iceland; and Ola Landgren, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Paul W
authors.initials: PW
authors.lastname: Dickman
authors.affiliation: Malin Hultcrantz, Sigurdur Y. Kristinsson, Åsa R. Derolf, and Magnus Björkholm, Karolinska University Hospital; Malin Hultcrantz, Sigurdur Y. Kristinsson, Therese M.-L. Andersson, Åsa R. Derolf, Sandra Eloranta, Paul W. Dickman, Paul C. Lambert, and Magnus Björkholm, Karolinska Institutet; Jan Samuelsson, South Hospital, Stockholm, Sweden; Sally R. Wilkes, University of Nottingham, Nottingham; Paul C. Lambert, University of Leicester, Leicester, United Kingdom; Sigurdur Y. Kristinsson, University of Iceland and Landspitali National University Hospital, Reykjavik, Iceland; and Ola Landgren, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Paul C
authors.initials: PC
authors.lastname: Lambert
authors.affiliation: Malin Hultcrantz, Sigurdur Y. Kristinsson, Åsa R. Derolf, and Magnus Björkholm, Karolinska University Hospital; Malin Hultcrantz, Sigurdur Y. Kristinsson, Therese M.-L. Andersson, Åsa R. Derolf, Sandra Eloranta, Paul W. Dickman, Paul C. Lambert, and Magnus Björkholm, Karolinska Institutet; Jan Samuelsson, South Hospital, Stockholm, Sweden; Sally R. Wilkes, University of Nottingham, Nottingham; Paul C. Lambert, University of Leicester, Leicester, United Kingdom; Sigurdur Y. Kristinsson, University of Iceland and Landspitali National University Hospital, Reykjavik, Iceland; and Ola Landgren, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Magnus
authors.initials: M
authors.lastname: Björkholm
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002318:Cardiovascular Diseases / Q000175:diagnosis / Q000401:mortality*; D016022:Case-Control Studies; D002423:Cause of Death; D003141:Communicable Diseases / Q000175:diagnosis / Q000401:mortality*; D005260:Female; D019337:Hematologic Neoplasms / Q000175:diagnosis / Q000401:mortality*; D006801:Humans; D008017:Life Expectancy; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000175:diagnosis / Q000401:mortality*; D011379:Prognosis; D012042:Registries; D018570:Risk Assessment; D012307:Risk Factors; D013548:Sweden / Q000453:epidemiology; D013997:Time Factors; D055815:Young Adult