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Intracranial haemorrhage in the Italian population of haemophilia patients with and without inhibitors
Author(s) -
ZA E.,
IORIO A.,
ROCINO A.,
ARTONI A.,
SANTORO R.,
TAGLIAFERRI A.,
COPPOLA A.,
CASTAMAN G.,
MANNUCCI P. M.
Publication year - 2012
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1111/j.1365-2516.2011.02611.x
Subject(s) - medicine , haemophilia , haemophilia a , hazard ratio , incidence (geometry) , pediatrics , univariate analysis , retrospective cohort study , intracranial haemorrhage , cumulative incidence , population , cohort , multivariate analysis , surgery , confidence interval , complication , physics , environmental health , optics
Summary. Intracranial haemorrhage (ICH) is the most serious bleeding symptom in haemophiliacs, resulting in high rates of mortality and disabling sequelae. The Association of Italian Haemophilia Centres carried out a retrospective survey (1987–2008) of ICH occurring in haemophiliacs with the goals to establish: (i) incidence, location of bleeding, death rate and disabling sequels; (ii) risk factors for ICH; and (iii) treatment used during the acute phase of ICH and for recurrence prevention. A total of 112 ICH episodes had occurred in 88 patients (78 haemophilia A, 10 haemophilia B), 24 of whom experienced recurrences. The cumulative hazard of ICH for the whole cohort over the entire follow‐up period was 26.7 per 1000 patients, and the annualized rate of ICH was 2.50 events per 1000 patients (95% CI 1.90–3.31). The risk of ICH was higher in the youngest children (24.4 per 1000, 95% CI 12.7–47.0 in the first year of age and 14.9, 95% CI 7.1–31.4 in the second year of age) and then progressively rose again after the age of 40. Univariate, bivariate (age‐adjusted) and multivariate analysis investigating the effects of patient characteristics on ICH occurrence showed that haemophilia severity and inhibitor status were strongly associated with ICH [severe vs. mild, HR 3.96 (2.39–6.57); inhibitor vs. non‐inhibitor 2.52 (1.46–4.35)]. HCV infection was also associated with the risk of ICH [HR 1.83 (1.25–2.69)]. Therapeutic suggestions based upon our experience to control ICH recurrence are provided.