Early Mortality in Paroxysmal Nocturnal Hemoglobinuria.
cohort study
comorbidity
epidemiology
mortality
paroxysmal nocturnal hemoglobinuria
pnh
survival
Journal
Cureus
ISSN: 2168-8184
Titre abrégé: Cureus
Pays: United States
ID NLM: 101596737
Informations de publication
Date de publication:
Oct 2023
Oct 2023
Historique:
accepted:
12
10
2023
medline:
29
11
2023
pubmed:
29
11
2023
entrez:
29
11
2023
Statut:
epublish
Résumé
The elevated mortality risk among patients with paroxysmal nocturnal hemoglobinuria (PNH) has been suggested to derive from a high risk of thromboembolism (TE); however, the risks of coexisting cardiovascular risk factors are not well described. We studied mortality associated with PNH taking comorbidity and treatment into account. Patients with PNH (n=115) were identified in the 1977-2016 Danish National Patient Register (DNPR). For each patient with PNH, we identified 50 age- and sex-matched general population comparators. Using the Kaplan-Meier estimator and Cox regression, we compared the overall survival of patients with comparators. Cumulative incidences were used to analyze the effects of comorbidity and the causes of death. One-year survival among patients and comparators was 92.2% and 99.4%, and after 10 years, it was 68.4% and 85.8%, respectively. Early mortality was associated with older age, higher levels of comorbidity, and solid malignancies prior to PNH diagnosis. The leading causes of death were infections and associated hematological diseases. Patients with early mortality were less likely to have received treatment with eculizumab and/or warfarin. Cardiovascular risk factors were evenly distributed between patients and comparators at diagnosis. We conclude that early mortality in PNH is associated with older age, cardiovascular comorbidity, and hematological malignancies.
Identifiants
pubmed: 38022316
doi: 10.7759/cureus.47225
pmc: PMC10653694
doi:
Types de publication
Journal Article
Langues
eng
Pagination
e47225Informations de copyright
Copyright © 2023, Sørensen et al.
Déclaration de conflit d'intérêts
The authors have declared that no competing interests exist.
Références
Eur J Cancer Prev. 2005 Jun;14(3):201-6
pubmed: 15901987
Br J Haematol. 2013 Jul;162(1):62-73
pubmed: 23617322
PLoS Med. 2015 Oct 06;12(10):e1001885
pubmed: 26440803
JAMA. 2005 Apr 6;293(13):1653-62
pubmed: 15811985
Eur J Haematol. 2021 Aug;107(2):211-218
pubmed: 34060690
Scand J Public Health. 2011 Jul;39(7 Suppl):30-3
pubmed: 21775347
Scand J Public Health. 2011 Jul;39(7 Suppl):26-9
pubmed: 21775346
Br J Haematol. 1992 Mar;80(3):399-405
pubmed: 1374629
Am J Hematol. 2019 Oct;94(10):1081-1090
pubmed: 31292991
Eur J Haematol. 2014 Oct;93(4):309-19
pubmed: 24758317
Clin Epidemiol. 2015 Feb 12;7:195-9
pubmed: 25709502
Blood. 2011 Jun 23;117(25):6786-92
pubmed: 21460245
Blood. 2013 Jun 20;121(25):4985-96; quiz 5105
pubmed: 23610373
Clin Epidemiol. 2018 Oct 12;10:1503-1508
pubmed: 30349397
Medicine (Baltimore). 2004 May;83(3):193-207
pubmed: 15118546
Epidemiol Infect. 2018 Nov;146(15):1965-1967
pubmed: 29843835
Blood. 2008 Oct 15;112(8):3099-106
pubmed: 18535202
Clin Epidemiol. 2015 Nov 17;7:449-90
pubmed: 26604824
Eur J Epidemiol. 2014 Aug;29(8):541-9
pubmed: 24965263
N Engl J Med. 1995 Nov 9;333(19):1253-8
pubmed: 7566002
Blood. 2014 Oct 30;124(18):2804-11
pubmed: 25237200
Clin Epidemiol. 2020 May 22;12:497-508
pubmed: 32547241
Clin Epidemiol. 2016 Jun 24;8:241-52
pubmed: 27445504
Scand J Public Health. 2011 Jul;39(7 Suppl):38-41
pubmed: 21775349
Int J Epidemiol. 2012 Jun;41(3):861-70
pubmed: 22253319
Blood. 2009 Jun 25;113(26):6522-7
pubmed: 19372253