Intracranial haemorrhage in the Italian population of haemophilia patients with and without inhibitors

E. Zanon, A. Iorio, A. Rocino, A. Artoni, R. Santoro, A. Tagliaferri, A. Coppola, G. Castaman, P. M. Mannucci, Giovanni Barillari, Alfredo Dragani, Gabriella Gamba, Anna Giuffrida, Mario Lapecorella, Giacomo Mancuso, Liguori Lucia, Maria Gabriella Mazzucconi, Maria Messina, Roberto Musso, Francesco De MartisGina Rossetti, Piercarla Schinco, Luca Spiezia, Lelia Valdré

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)39-45
Number of pages7
Issue number1
Publication statusPublished - Jan 2012


  • FVIII inhibitor
  • Haemophilia A
  • Haemophilia B
  • Intracranial haemorrhage

ASJC Scopus subject areas

  • Hematology
  • Genetics(clinical)


Dive into the research topics of 'Intracranial haemorrhage in the Italian population of haemophilia patients with and without inhibitors'. Together they form a unique fingerprint.

Cite this