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Mortality caused by intracranial bleeding in non-severe hemophilia A patients

Loomans, J. I., Eckhardt, C. L., Reitter-Pfoertner, S. E., Holmstrom, M., Van Gorkom, B. L., Leebeek, F. W. G., Santoro, C., Haya, S., Meijer, K., Nijziel, M. R., Van Der Bom, J. G. & Fijnvandraat, K., Jun-2017, In : JOURNAL OF THROMBOSIS AND HAEMOSTASIS. 15, 6, p. 1115-1122 8 p.

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  • Mortality caused by intracranial bleeding in non-severe hemophilia A patients

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DOI

  • Janneke I. Loomans
  • Corien L. Eckhardt
  • Sylvia E. Reitter-Pfoertner
  • Mats Holmstrom
  • B. Laros Van Gorkom
  • F. W. G. Leebeek
  • C. Santoro
  • Saturnino Haya
  • K. Meijer
  • M. R. Nijziel
  • J. G. Van Der Bom
  • K. Fijnvandraat

Background: Non-severe hemophilia (factor VIII concentration [FVIII: C] of 2-40 IU dL(-1)) is characterized by a milder bleeding phenotype than severe hemophilia A. However, some patients with non-severe hemophilia A suffer from severe bleeding complications that may result in death. Data on bleeding-related causes of death, such as fatal intracranial bleeding, in non-severe patients are scarce. Such data may provide new insights into areas of care that can be improved. Aims: To describe mortality rates, risk factors and comorbidities associated with fatal intracranial bleeding in non-severe hemophilia A patients. Methods: We analyzed data from the INSIGHT study, an international cohort study of all non-severe hemophilia A patients treated with FVIII concentrates during the observation period between 1980 and 2010 in 34 participating centers across Europe and Australia. Clinical data and vital status were collected from 2709 patients. We report the standardized mortality rate for patients who suffered from fatal intracranial bleeding, using a general European male population as a control population. Results: Twelve per cent of the 148 deceased patients in our cohort of 2709 patients died from intracranial bleeding. The mortality rate between 1996 and 2010 for all ages was 3.5-fold higher than that in the general population (95% confidence interval [CI] 2.0-5.8). Patients who died from intracranial bleeding mostly presented with mild hemophilia without clear comorbidities. Conclusion: Non-severe hemophilia A patients have an increased risk of dying from intracranial bleeding in comparison with the general population. This demonstrates the need for specialized care for non-severe hemophilia A patients.

Original languageEnglish
Pages (from-to)1115-1122
Number of pages8
JournalJOURNAL OF THROMBOSIS AND HAEMOSTASIS
Volume15
Issue number6
Publication statusPublished - Jun-2017

    Keywords

  • cohort study, epidemiology, hemophilia A, intracranial hemorrhage, mortality, CRITICAL-CARE MANAGEMENT, INTRACEREBRAL HEMORRHAGE, INHIBITOR DEVELOPMENT, FACTOR-VIII, DEATH, SURVIVAL, COHORT, RISK

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