Abstract Number: PB1040
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical
Background: The most serious bleeding event in patients with haemophilia (PWHs) is certainly intracranial haemorrhage (ICH) which leads to disability and in some cases to death. Children ≤2 years and adults ≥50 years are the subjects most at risk for ICH.
Aims: To evaluate incidence, mortality, management and risk factors for ICH in an Italian population of PWHs
Methods: All ICHs in PWH were retrospectively and prospectively collected at 13 Italian Haemophilia Centres. All data of patients, ICHs management and outcomes were evaluated from 2009 to 2018.
Results: Overall 44 ICHs were found in 4990 haemophiliacs. ICH occurred in 10/15 children ≤ 2 yrs, none of them were on prophylaxis at the time of bleeding; one ICH occurred in a 4-year-old child during ITI. ICHs occurred in 29 adults, 44.8% with mild haemophilia. Overall patients with severe haemophilia were 61.4%; 100.0% (15/15) of children. Only 7/44 patients received a prophylactic regimen before ICH. Inhibitors were present in 13.6% of patients. 15/29 adult PWHs suffered from hypertension, 84.6% were mild subjects and 25.0% were moderate to severe (p< 0.05). ICH was spontaneous in the 69.8%. Surgery was required in 19/44 patients for cerebral hematoma evacuation. Patients treated with FVIII/FIX concentrates or rFVIIa for at least three weeks were 76.7%. Subjects who died before starting or during treatment were 31.8%. Of the survivors 43.3% became permanently disabled. Only one-third of patients continued the prophylaxis after the acute treatment.
Conclusions: The final results obtained by our Registry confirm a high incidence of ICH in infants ≤2 years and in adults ≥50 years, especially in mild adult PWHs presenting concomitant hypertension. The majority of PWHs were treated on-demand during ICH, which indicates the important role of prophylaxis. Perhaps the use of new subcutaneous agents, as emicizumab, could be help to prevent the risk of ICH especially in children.
To cite this abstract in AMA style:
Zanon E, Pasca S, Demartis F, Tagliaferri A, Santoro C, Cantori I, Molinari AC, Biasioli C, Coppola A, Castaman G, Linari S, Luciani M, Sottilotta G, Ricca I, Pollio B, Borchiellini A, Santagostino E, Simioni P. Is intracranial haemorrhage in haemophilia patients still a present risk in the third millennium? The final results of the EMO.REC Registry: a 10-year Italian study on intracranial haemorrhage in haemophilia patients [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/is-intracranial-haemorrhage-in-haemophilia-patients-still-a-present-risk-in-the-third-millennium-the-final-results-of-the-emo-rec-registry-a-10-year-italian-study-on-intracranial-haemorrhage-in-haem/. Accessed March 21, 2024.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/is-intracranial-haemorrhage-in-haemophilia-patients-still-a-present-risk-in-the-third-millennium-the-final-results-of-the-emo-rec-registry-a-10-year-italian-study-on-intracranial-haemorrhage-in-haem/