Intracranial Hemorrhage in Hemophilia - A Systematic Review
ISTH Academy. Zwagemaker A. Jul 10, 2019; 274141; OC 70.3 Topic: Hemophilia - Clinical
AnneFleur Zwagemaker
AnneFleur Zwagemaker
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OC 70.3

Intracranial Hemorrhage in Hemophilia - A Systematic Review

A. Zwagemaker1, C. Vuong1, J. Jansen1, S. Gouw2, M. Coppens3, J. van der Bom4, K. Fijnvandraat1
1Amsterdam UMC, University of Amsterdam, Pediatric Hematology, Amsterdam, the Netherlands, 2Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands, 3Amsterdam UMC, University of Amsterdam, Vascular Medicine, Amsterdam, the Netherlands, 4Leiden University Medical Center, Clinical Epidemiology, Leiden, the Netherlands

Main Topic: Hemophilia and Bleeding (including Transfusion)
Category: Hemophilia - Clinical

Background: Intracranial hemorrhage (ICH) is a life-threatening event in hemophilia. While the focus of most studies in hemophilia patients is joint bleeding, various studies have assessed ICH in hemophilia, reporting a wide range of incidence and mortality rates. More precise estimates of ICH numbers in hemophilia are important to aid in identification of patients at risk and to guide future prevention.
Aims: We conduct a systematic review to assess incidence and mortality of ICH in patients with hemophilia.
Methods: MEDLINE and EMBASE were searched by use of key terms related to hemophilia, ICH and mortality. Studies reporting on a representative congenital hemophilia population were included. Screening, data collection and critical appraisal was performed by two independent reviewers. Random-effects pooled rates are calculated for the whole population and for specified subgroups according to disease severity, age, treatment regimen, historical time period and geographic location. The full protocol can be found at PROSPERO (record ID 100278).
Results: The search yielded 2058 de-duplicated hits on April 12, 2018. We analyzed 56 relevant studies that described 82527 hemophilia patients and 1508 ICH events over 6 decades (mid-year range 1960 to 2014). ICH incidence rate ranged between 21 and 2752 per 100.000 person-years, with a pooled rate of 400 per 100.000 person-years. Over the past decades, the pooled ICH incidence in hemophilia patients declined from around 8% in the period 1960-1979 to 3% in 2000-2018.
Conclusions: In the general population, the incidence of hemorrhagic stroke is estimated at 25 per 100.000 person-years. This is lower than the pooled incidence of 400 per 100.000 person-years that was found for hemophilia patients. At ISTH 2019, we will present our results on ICH in hemophilia in relation to specific subgroups at high risk.

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