Mortality, life expectancy, and causes of death of persons with hemophilia in the Netherlands 2001-2018

Shermarke Hassan; Rory C. Monahan; Evelien P. Mauser-Bunschoten; Lize F. D. van Vulpen; Jeroen Eikenboom; Erik A. M. Beckers; Louise Hooimeijer; Paula F. Ypma; Laurens Nieuwenhuizen; Michiel Coppens; Saskia E. M. Schols; Frank W. G. Leebeek; Cees Smit; Mariette H. Driessens; Saskia le Cessie; Erna C. van Balen; Frits R. Rosendaal; Johanna G. van Der Bom; Samantha C. Gouw

doi:10.1111/jth.15182

Mortality, life expectancy, and causes of death of persons with hemophilia in the Netherlands 2001-2018

Shermarke Hassan, Rory C. Monahan, Evelien P. Mauser-Bunschoten, Lize F. D. van Vulpen, Jeroen Eikenboom, Erik A. M. Beckers, Louise Hooimeijer, Paula F. Ypma, Laurens Nieuwenhuizen, Michiel Coppens, Saskia E. M. Schols, Frank W. G. Leebeek, Cees Smit, Mariette H. Driessens, Saskia le Cessie, Erna C. van Balen, Frits R. Rosendaal, Johanna G. van Der Bom, Samantha C. Gouw^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

Background Treatment of patients with hemophilia has advanced over the past decades, but it is unknown whether this has resulted in a normal life expectancy in the Netherlands.

Objective This observational cohort study aimed to assess all-cause and cause-specific mortality in patients with hemophilia in the Netherlands between 2001 and 2018 and to compare mortality and life expectancy with previous survival assessments from 1973 onward.

Patients/methods All 1066 patients with hemophilia who participated in a nationwide survey in 2001 were followed until July 2018.

Results Information on 1031 individuals (97%) was available, of whom 142 (14%) deceased during follow-up. Compared with the general Dutch male population, mortality of patients with hemophilia was still increased (standardized mortality ratio: 1.4, 95% confidence interval: 1.2-1.7). Intracranial bleeding and malignancies were the most common causes of death. Estimated median life expectancy of patients with hemophilia was 77 years, 6 years lower than the median life expectancy of the general Dutch male population (83 years). Over the past 45 years, death rates of patients with hemophilia have consistently decreased, approaching the survival experience of the general population. Over the past decades, mortality due to human immunodeficiency virus and hepatitis C virus infections has decreased, death due to intracranial hemorrhages has increased, and death due to ischemic heart disease has remained consistently low over time.

Conclusions Survival in patients with hemophilia in the Netherlands has improved over time but is still lower than that of the general population.

Original language	English
Pages (from-to)	645-653
Number of pages	9
Journal	Journal of Thrombosis and Haemostasis
Volume	19
Issue number	3
Early online date	18 Dec 2020
DOIs	https://doi.org/10.1111/jth.15182
Publication status	Published - Mar 2021

Keywords

hemophilia
hepatitis C
HIV
life expectancy
mortality
INTRACRANIAL HEMORRHAGE
CARDIOVASCULAR-DISEASE
LIVER-DISEASE
DUTCH
COHORT
POPULATION
PREVALENCE
ANTIBODIES
MANAGEMENT
CANCERS

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10.1111/jth.15182Licence: CC BY-NC

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Hassan, S., Monahan, R. C., Mauser-Bunschoten, E. P., van Vulpen, L. F. D., Eikenboom, J., Beckers, E. A. M., Hooimeijer, L., Ypma, P. F., Nieuwenhuizen, L., Coppens, M., Schols, S. E. M., Leebeek, F. W. G., Smit, C., Driessens, M. H., le Cessie, S., van Balen, E. C., Rosendaal, F. R., van Der Bom, J. G., & Gouw, S. C. (2021). Mortality, life expectancy, and causes of death of persons with hemophilia in the Netherlands 2001-2018. Journal of Thrombosis and Haemostasis, 19(3), 645-653. https://doi.org/10.1111/jth.15182

@article{3623496db2d04975bc5af2f19eebdf90,

title = "Mortality, life expectancy, and causes of death of persons with hemophilia in the Netherlands 2001-2018",

abstract = "Background Treatment of patients with hemophilia has advanced over the past decades, but it is unknown whether this has resulted in a normal life expectancy in the Netherlands.Objective This observational cohort study aimed to assess all-cause and cause-specific mortality in patients with hemophilia in the Netherlands between 2001 and 2018 and to compare mortality and life expectancy with previous survival assessments from 1973 onward.Patients/methods All 1066 patients with hemophilia who participated in a nationwide survey in 2001 were followed until July 2018.Results Information on 1031 individuals (97%) was available, of whom 142 (14%) deceased during follow-up. Compared with the general Dutch male population, mortality of patients with hemophilia was still increased (standardized mortality ratio: 1.4, 95% confidence interval: 1.2-1.7). Intracranial bleeding and malignancies were the most common causes of death. Estimated median life expectancy of patients with hemophilia was 77 years, 6 years lower than the median life expectancy of the general Dutch male population (83 years). Over the past 45 years, death rates of patients with hemophilia have consistently decreased, approaching the survival experience of the general population. Over the past decades, mortality due to human immunodeficiency virus and hepatitis C virus infections has decreased, death due to intracranial hemorrhages has increased, and death due to ischemic heart disease has remained consistently low over time.Conclusions Survival in patients with hemophilia in the Netherlands has improved over time but is still lower than that of the general population.",

keywords = "hemophilia, hepatitis C, HIV, life expectancy, mortality, INTRACRANIAL HEMORRHAGE, CARDIOVASCULAR-DISEASE, LIVER-DISEASE, DUTCH, COHORT, POPULATION, PREVALENCE, ANTIBODIES, MANAGEMENT, CANCERS",

author = "Shermarke Hassan and Monahan, {Rory C.} and Mauser-Bunschoten, {Evelien P.} and {van Vulpen}, {Lize F. D.} and Jeroen Eikenboom and Beckers, {Erik A. M.} and Louise Hooimeijer and Ypma, {Paula F.} and Laurens Nieuwenhuizen and Michiel Coppens and Schols, {Saskia E. M.} and Leebeek, {Frank W. G.} and Cees Smit and Driessens, {Mariette H.} and {le Cessie}, Saskia and {van Balen}, {Erna C.} and Rosendaal, {Frits R.} and {van Der Bom}, {Johanna G.} and Gouw, {Samantha C.}",

note = "Funding Information: SH, RCM, EPMB, LFDvV, EAMB, LH, PFY, LN, SEMS, CS, MHD, SLC, ECvB, FRR, and SCG have no interests to disclose. JE has received research support from CSL Behring for research outside this project. MC has received financial support for research, as well as lecturing and consultancy fees, from Bayer, CSL Behring, Pfizer, Sanquin Blood Supply, and UniQure. FWGL has received an unrestricted grant from CSL Behring and Shire/Takeda for studies on von Willebrand disease. He is a consultant for UniQure, NovoNordisk, and Shire/Takeda, of which the fees go to the university. He is member of a DSMB for a study from Roche. JGvdB Has been a teacher on educational activities of Bayer. Publisher Copyright: {\textcopyright} 2020 The Authors. Journal of Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis",

year = "2021",

month = mar,

doi = "10.1111/jth.15182",

language = "English",

volume = "19",

pages = "645--653",

journal = "Journal of Thrombosis and Haemostasis",

issn = "1538-7933",

publisher = "Wiley",

number = "3",

}

Hassan, S, Monahan, RC, Mauser-Bunschoten, EP, van Vulpen, LFD, Eikenboom, J, Beckers, EAM, Hooimeijer, L, Ypma, PF, Nieuwenhuizen, L, Coppens, M, Schols, SEM, Leebeek, FWG, Smit, C, Driessens, MH, le Cessie, S, van Balen, EC, Rosendaal, FR, van Der Bom, JG & Gouw, SC 2021, 'Mortality, life expectancy, and causes of death of persons with hemophilia in the Netherlands 2001-2018', Journal of Thrombosis and Haemostasis, vol. 19, no. 3, pp. 645-653. https://doi.org/10.1111/jth.15182

TY - JOUR

T1 - Mortality, life expectancy, and causes of death of persons with hemophilia in the Netherlands 2001-2018

AU - Hassan, Shermarke

AU - Monahan, Rory C.

AU - Mauser-Bunschoten, Evelien P.

AU - van Vulpen, Lize F. D.

AU - Eikenboom, Jeroen

AU - Beckers, Erik A. M.

AU - Hooimeijer, Louise

AU - Ypma, Paula F.

AU - Nieuwenhuizen, Laurens

AU - Coppens, Michiel

AU - Schols, Saskia E. M.

AU - Leebeek, Frank W. G.

AU - Smit, Cees

AU - Driessens, Mariette H.

AU - le Cessie, Saskia

AU - van Balen, Erna C.

AU - Rosendaal, Frits R.

AU - van Der Bom, Johanna G.

AU - Gouw, Samantha C.

N1 - Funding Information: SH, RCM, EPMB, LFDvV, EAMB, LH, PFY, LN, SEMS, CS, MHD, SLC, ECvB, FRR, and SCG have no interests to disclose. JE has received research support from CSL Behring for research outside this project. MC has received financial support for research, as well as lecturing and consultancy fees, from Bayer, CSL Behring, Pfizer, Sanquin Blood Supply, and UniQure. FWGL has received an unrestricted grant from CSL Behring and Shire/Takeda for studies on von Willebrand disease. He is a consultant for UniQure, NovoNordisk, and Shire/Takeda, of which the fees go to the university. He is member of a DSMB for a study from Roche. JGvdB Has been a teacher on educational activities of Bayer. Publisher Copyright: © 2020 The Authors. Journal of Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis

PY - 2021/3

Y1 - 2021/3

N2 - Background Treatment of patients with hemophilia has advanced over the past decades, but it is unknown whether this has resulted in a normal life expectancy in the Netherlands.Objective This observational cohort study aimed to assess all-cause and cause-specific mortality in patients with hemophilia in the Netherlands between 2001 and 2018 and to compare mortality and life expectancy with previous survival assessments from 1973 onward.Patients/methods All 1066 patients with hemophilia who participated in a nationwide survey in 2001 were followed until July 2018.Results Information on 1031 individuals (97%) was available, of whom 142 (14%) deceased during follow-up. Compared with the general Dutch male population, mortality of patients with hemophilia was still increased (standardized mortality ratio: 1.4, 95% confidence interval: 1.2-1.7). Intracranial bleeding and malignancies were the most common causes of death. Estimated median life expectancy of patients with hemophilia was 77 years, 6 years lower than the median life expectancy of the general Dutch male population (83 years). Over the past 45 years, death rates of patients with hemophilia have consistently decreased, approaching the survival experience of the general population. Over the past decades, mortality due to human immunodeficiency virus and hepatitis C virus infections has decreased, death due to intracranial hemorrhages has increased, and death due to ischemic heart disease has remained consistently low over time.Conclusions Survival in patients with hemophilia in the Netherlands has improved over time but is still lower than that of the general population.

AB - Background Treatment of patients with hemophilia has advanced over the past decades, but it is unknown whether this has resulted in a normal life expectancy in the Netherlands.Objective This observational cohort study aimed to assess all-cause and cause-specific mortality in patients with hemophilia in the Netherlands between 2001 and 2018 and to compare mortality and life expectancy with previous survival assessments from 1973 onward.Patients/methods All 1066 patients with hemophilia who participated in a nationwide survey in 2001 were followed until July 2018.Results Information on 1031 individuals (97%) was available, of whom 142 (14%) deceased during follow-up. Compared with the general Dutch male population, mortality of patients with hemophilia was still increased (standardized mortality ratio: 1.4, 95% confidence interval: 1.2-1.7). Intracranial bleeding and malignancies were the most common causes of death. Estimated median life expectancy of patients with hemophilia was 77 years, 6 years lower than the median life expectancy of the general Dutch male population (83 years). Over the past 45 years, death rates of patients with hemophilia have consistently decreased, approaching the survival experience of the general population. Over the past decades, mortality due to human immunodeficiency virus and hepatitis C virus infections has decreased, death due to intracranial hemorrhages has increased, and death due to ischemic heart disease has remained consistently low over time.Conclusions Survival in patients with hemophilia in the Netherlands has improved over time but is still lower than that of the general population.

KW - hemophilia

KW - hepatitis C

KW - HIV

KW - life expectancy

KW - mortality

KW - INTRACRANIAL HEMORRHAGE

KW - CARDIOVASCULAR-DISEASE

KW - LIVER-DISEASE

KW - DUTCH

KW - COHORT

KW - POPULATION

KW - PREVALENCE

KW - ANTIBODIES

KW - MANAGEMENT

KW - CANCERS

U2 - 10.1111/jth.15182

DO - 10.1111/jth.15182

M3 - Article

C2 - 33217158

SN - 1538-7933

VL - 19

SP - 645

EP - 653

JO - Journal of Thrombosis and Haemostasis

JF - Journal of Thrombosis and Haemostasis

IS - 3

ER -