Inhibitor development and mortality in non-severe hemophilia A

Autor: C.L. Eckhardt, J.I. Loomans, A.S. van Velzen, M. Peters, E.P. Mauser‐Bunschoten, R. Schwaab, M.G. Mazzucconi, A. Tagliaferri, B. Siegmund, S.E. Reitter‐Pfoertner, J.G. van der Bom, K. Fijnvandraat, P.W. Kamphuisen, K. Peerlinck, J. Oldenburg, E. Santagostino, J. Astermark, C.L Eckhardt, A.S van Velzen, N. Streefkerk, J.L. Loomans, A. van Eijkelenburg, A.J. Jansen, C.C. Kruijt, B. van Tienoven, A.C.G. van Baar, I.W. Corten, K. Meijer, M.R. Nijziel, N. Dors, K. Hamulyak, E. Beckers, P.P. Brons, B.A.P. Laros‐van Gorkom, W.L. van Heerde, F. Leebeek, M. Kruip, M.H. Cnossen, E. Mauser‐Bunschoten, K. Fischer, F.J. Smiers, C. Hermans, R. Klamroth, C. Escuriola‐Ettingshausen, C. Königs, P. Petrini, M. Holmström, A. Mäkipernaa, C. Male, I. Pabinger, R.D. Keenan, R. Liesner, K. Khair, T.T. Yee, D.P. Hart, S. Rangarajan, M. Mitchell, G. Thompson, S. Haya, A. Moret, A.R. Cid, V. Jimenez‐Yuste, M.E. Mancuso, M.G. Mazzuconni, C. Santoro, M. Morfini, G. Castaman, P. Schinco, G.F. Rivolta, H. Platokouki, S. McRae
Přispěvatelé: Cardiovascular Centre (CVC), Vascular Ageing Programme (VAP), ACS - Amsterdam Cardiovascular Sciences, AII - Amsterdam institute for Infection and Immunity, General Paediatrics, Other departments, Other Research, Paediatric Infectious Diseases / Rheumatology / Immunology, CCA -Cancer Center Amsterdam, Graduate School, Paediatric Oncology, Gastroenterology and Hepatology
Jazyk: angličtina
Rok vydání: 2015
Předmět:
Adult
Male
medicine.medical_specialty
Time Factors
Adolescent
Vascular damage Radboud Institute for Health Sciences [Radboudumc 16]
Population
Hemorrhage
Cause of death
Rare cancers Radboud Institute for Molecular Life Sciences [Radboudumc 9]
Research Support
Severe hemophilia A
Hemophilia A
Mortality
Neutralizing antibodies
Hematology
Risk Assessment
Severity of Illness Index
Young Adult
Interquartile range
Risk Factors
Internal medicine
medicine
Journal Article
Humans
Non-U.S. Gov't
education
Child
Aged
Autoantibodies
Retrospective Studies
education.field_of_study
Factor VIII
business.industry
Mortality rate
Research Support
Non-U.S. Gov't
Australia
Retrospective cohort study
Middle Aged
Antibodies
Neutralizing
Confidence interval
Multicenter Study
Europe
Life expectancy
business
Biomarkers
Zdroj: Journal of Thrombosis and Haemostasis, 13(7), 1217-1225. Wiley
Journal of Thrombosis and Haemostasis, 13(7), 1217-1225
Journal of thrombosis and haemostasis, 13(7), 1217-1225. Wiley-Blackwell
Journal of Thrombosis and Haemostasis, 13, 1217-25
Journal of Thrombosis and Haemostasis, 13, 7, pp. 1217-25
Journal of Thrombosis and Haemostasis, 13(7), 1217. Wiley-Blackwell
ISSN: 1538-7933
Popis: BACKGROUND: The life expectancy of non-severe hemophilia A (HA) patients equals the life expectancy of the non-hemophilic population. However, data on the effect of inhibitor development on mortality and on hemophilia-related causes of death are scarce. The development of neutralizing factor VIII antibodies in non-severe HA patients may dramatically change their clinical outcome due to severe bleeding complications.OBJECTIVES: We assessed the association between the occurrence of inhibitors and mortality in patients with non-severe HA.METHODS: In this retrospective cohort study, clinical data and vital status were collected for 2709 non-severe HA patients (107 with inhibitors) who were treated between 1980 and 2011 in 34 European and Australian centers. Mortality rates for patients with and without inhibitors were compared.RESULTS: During 64,200 patient-years of follow-up, 148 patients died (mortality rate, 2.30 per 1000 person-years; 95% confidence interval (CI), 1.96-2.70) at a median age of 64 years (interquartile range [IQR], 49-76). In 62 patients (42%) the cause of death was hemophilia related. Sixteen inhibitor patients died at a median age of 71 years (IQR, 60-81). In ten patients the inhibitor was present at time of death; seven of them died of severe bleeding complications. The all-cause mortality rate in inhibitor patients was > 5 times increased compared with that for those without inhibitors (age-adjusted mortality rate ratio, 5.6).CONCLUSION: Inhibitor development in non-severe hemophilia is associated with increased mortality. High rates of hemophilia-related mortality in this study indicate that non-severe hemophilia is not mild at all and stress the importance of close follow-up for these patients.
Databáze: OpenAIRE
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