Incidence and mortality rates of intracranial hemorrhage in hemophilia: a systematic review and meta-analysis

Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands. Amsterdam University Medical Centers, Amsterdam, Netherlands. Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. Nanfang Hospital, Guangzhou, China. Inha University Hospital, Incheon, Korea, Republic of. Sanquin Research & Leiden University Medical Center, Leiden, Netherlands. Amsterdam UMC, Amsterdam, Netherlands.

Blood. 2021
PICO Summary

Population

Persons with haemophilia of all ages (45 studies, n= 54,470).

Intervention

Summarized evidence on intracranial haemorrhage (ICH) incidence and calculated pooled ICH incidence and mortality with a systematic review and meta-analysis.

Comparison

Outcome

Incidence and mortality were pooled with a Poisson-normal model or a binomial-normal model. The included studies represented 54,470 patients, 809,151 person-years, and 5,326 live births of patients with haemophilia. In persons of all ages, the pooled ICH incidence and mortality rates were 2.3 (95% confidence interval (CI): 1.2-4.8) and 0.8 (95% CI: 0.5-1.2) per 1,000 person-years, respectively. In children and young adults, the pooled ICH incidence and mortality rates were 7.4 (95% CI: 4.9-11.1) and 0.5 (95% CI: 0.3-0.9) per 1,000 person-years, respectively. In neonates, the pooled cumulative ICH incidence was 2.1% (95% CI: 1.5-2.8) per 100 live births. ICH was classified as spontaneous in 35% to 58% of cases.
Abstract
Intracranial hemorrhage (ICH) is a severe complication that is relatively common among hemophilia patients. This systematic review aimed to obtain more precise estimates of ICH incidence and mortality in hemophilia, which may be important for patients, caregivers, researchers and health policy-makers. PubMed and EMBASE were systematically searched using terms related to "hemophilia" and "intracranial hemorrhage" or "mortality". Studies that allowed calculation of ICH incidence or mortality rates in a hemophilia population of at least 50 patients were included. We summarized evidence on ICH incidence and calculated pooled ICH incidence and mortality in three age groups: (1) persons of all ages with hemophilia, (2) children and young adults below 25 years of age with hemophilia and (3) neonates with hemophilia. Incidence and mortality were pooled with a Poisson-Normal model or a Binomial-Normal model. We included 45 studies that represented 54 470 patients, 809 151 person-years and 5326 live births of hemophilia patients. In persons of all ages, the pooled ICH incidence and mortality rates were 2.3 (95% CI 1.2-4.8) and 0.8 (95% CI 0.5-1.2) per 1000 person-years, respectively. In children and young adults, the pooled ICH incidence and mortality rates were 7.4 (95% CI 4.9-11.1) and 0.5 (95% CI 0.3-0.9) per 1000 person-years, respectively. In neonates, the pooled cumulative ICH incidence was 2.1% (95% CI 1.5-2.8) per 100 live births. ICH was classified as spontaneous in 35-58% of cases. Our findings suggest that ICH is an important problem in hemophilia that occurs among all ages, requiring adequate preventive strategies.
Study details
Study Design : Systematic Review
Language : eng
Credits : Bibliographic data from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine