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. 2019 Oct 22;3(20):2980-2985.
doi: 10.1182/bloodadvances.2019000476.

Occurrence, thromboembolic risk, and mortality in Danish patients with cold agglutinin disease

Lauren C Bylsma  1 , Anne Gulbech Ording  2 , Adam Rosenthal  3 , Buket Öztürk  2 , Jon P Fryzek  1   2 , Jaime Morales Arias  3 , Alexander Röth  4 , Sigbjørn Berentsen  5
Affiliations

Occurrence, thromboembolic risk, and mortality in Danish patients with cold agglutinin disease

Lauren C Bylsma et al. Blood Adv. .

Abstract

Cold agglutinin disease (CAD) is a rare form of autoimmune hemolytic anemia with limited epidemiological and clinical data. We used the Danish National Patient Registries to examine CAD occurrence and risk of thromboembolic events (TEs) and mortality in CAD patients compared with a matched cohort from the general population in Denmark. We identified 72 patients diagnosed with CAD and 720 matched controls between 1999 and 2013. For 2013, the most recent year of study, crude incidence of CAD was 0.18 per 100 000 inhabitants per year and prevalence was 1.26 per 100 000 inhabitants. Risk of TEs was higher in the CAD patient cohort than in the comparison cohort at 1 year (7.2% of CAD patients had TEs vs 1.9% of comparisons), 3 years (9.0% vs 5.3%), and 5 years (11.5% vs 7.8%) after the index date. The median survival was 8.5 years. CAD patients had increased mortality compared with the general population cohort (adjusted hazard ratio [aHR], 1.84; 95% confidence interval [CI], 1.10-3.06; P = .020), with the highest mortality observed during the first 5 years after diagnosis (aHR, 2.27; 95% CI, 1.32-3.89; P = .003). Mortality rates 1 and 5 years after diagnosis were 17% and 39% in the CAD group vs 3% and 18% in the comparison cohort, respectively. CAD is a rare illness characterized by increased risk of TEs and mortality.

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Conflict of interest statement

Conflict-of-interest disclosure: S.B. has received research support from Mundipharma, lecture honoraria from Alexion, Sanofi, and Janssen-Cilag, and travel support from Alexion, Apellis, and Celgene and has been an advisory board member for Sanofi; L.C.B. and J.P.F. are employees of EpidStrategies, a Division of ToxStrategies, Inc.; A. Rosenthal and J.M.A. are employees of Sanofi; and A. Röth has received research support from Alexion and Roche, lecture honoraria from Alexion, Novartis, and Sanofi, and travel support from Alexion and has been an advisory board member for Alexion and Sanofi. The remaining authors declare no competing financial interests.

Figures

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Graphical abstract
Figure 1.
Figure 1.
Kaplan-Meier survival curve for CAD patients and matched general population comparison cohort.
See this image and copyright information in PMC

References

    1. Berentsen S. How I manage patients with cold agglutinin disease. Br J Haematol. 2018;181(3):320-330. - PubMed
    1. Swiecicki PL, Hegerova LT, Gertz MA. Cold agglutinin disease. Blood. 2013;122(7):1114-1121. - PubMed
    1. Berentsen S, Tjønnfjord GE. Diagnosis and treatment of cold agglutinin mediated autoimmune hemolytic anemia. Blood Rev. 2012;26(3):107-115. - PubMed
    1. Berentsen S. Cold agglutinin disease. Hematology Am Soc Hematol Educ Program. 2016;2016:226-231. - PMC - PubMed
    1. Shi J, Rose EL, Singh A, et al. . TNT003, an inhibitor of the serine protease C1s, prevents complement activation induced by cold agglutinins. Blood. 2014;123(26):4015-4022. - PubMed

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