High Impact and Severe Outcomes of Intracranial Hemorrhage (ICH) in an Italian Population of Adult Mild Hemophiliac Patients: Data from the EMO.REC Registry

E. Zanon¹, S. Pasca¹, F. Demartis², A. Tagliaferri³, C. Santoro⁴, I. Cantori⁵, A.C. Molinari⁶, C. Biasioli⁷, A. Coppola³, G. Castaman², S. Linari², M. Luciani⁸, G. Sottilotta⁹, I. Ricca¹⁰, B. Pollio¹⁰, A. Borchiellini¹¹, A. Tosetto¹², P. Simioni¹, F. Peyvandi¹³

¹Padua University Hospital, Padua, Italy, ²Careggi University Hospital, Florence, Italy, ³Parma University Hospital, Parma, Italy, ⁴Umberto I University Hospital, Rome, Italy, ⁵Hospital of Macerata, Macerata, Italy, ⁶Gaslini Children Hospital, Genoa, Italy, ⁷Cesena Hospital, Cesena, Italy, ⁸Bambin Gesù Children Hospital, Rome, Italy, ⁹Hospital of Reggio Calabria, Reggio Calabria, Italy, ¹⁰Regina Margherita Children Hospital, Turin, Italy, ¹¹Le Molinette Hospital, Turin, Italy, ¹²S. Bortolo Hospital, Vicenza, Italy, ¹³Milan University Hospital, Milan, Italy

Abstract Number: LPB0115

Meeting: ISTH 2021 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical

Background: Patients with mild hemophilia are usually considered to be at low-risk of bleeding, but the increase in mean age, associated with some concomitant diseases such as hypertension, and the absence of prophylactic treatment puts them at risk of severe or even fatal bleeding. To date, data in this regard are still scarce.

Aims: To assess the impact of ICH in an Italian population of adult mild patients with hemophilia (PWH) in terms of risk factors, incidence, treatments and outcomes.

Methods: All data of mild PWH with ICH were retrospectively/prospectively collected at 13 Italian Hemophilia Centers, and subsequently analyzed and compared with the moderate/severe subjects also present in our EMO.REC Registry.

Results: 31 adult patients with ICH were recorded, of whom 45.2% had mild hemophilia. The incidence/year x 1000 mild hemophilicacs was 0.24 , IC 95% (0.14-0.40). Mean age at diagnosis was 62.5years, higher than non-mild patients (45.0years). In 92.9% of cases, ICHs were spontaneous. Hypertension was reported in 85.7% of mild patients, while only in 23.5% of non-mild (p<0.05). At the time of ICH only 7.1% of mild subjects were on prophylaxis versus 29.4% of the moderate/severe. A treatment with factor concentrates was administered for at least three weeks to 78.5% of mild subjects. 50% of mild PWH underwent neurosurgery. Mortality rate in moderate-severe was almost twice as high as in mild subjects (52.9% vs 28.5%). Prophylaxis was maintained life-long only in 20% of mild survivors, 45.5% of mild survivors had a permanent disability. One on-demand patient had an ICH recurrence.

Conclusions: The impact of ICH in adult mild PWH is high, with severe outcomes. The correction of risk factors such as hypertension, is not enough to prevent an onset. The prophylaxis is not usual in mild hemophiliacs, innovative strategic therapies could be necessary to reduce the hemorrhagic incidence in this group of patients.

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, Tosetto A, Simioni P, Peyvandi F. High Impact and Severe Outcomes of Intracranial Hemorrhage (ICH) in an Italian Population of Adult Mild Hemophiliac Patients: Data from the EMO.REC Registry [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/high-impact-and-severe-outcomes-of-intracranial-hemorrhage-ich-in-an-italian-population-of-adult-mild-hemophiliac-patients-data-from-the-emo-rec-registry/. Accessed September 27, 2023.

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