Bleeding phenotype and correlation with factor XI (FXI) activity in congenital FXI deficiency: results of a retrospective study from a single centre

Summary Bleeding phenotype in factor XI (FXI)‐deficient patients is variable, and not related to baseline FXI:Act. Aims of our study were to describe the characteristics and the management of surgery and deliveries in FXI‐deficient patients, and to investigate the relationship between the haemorrhag...

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Published in:	Haemophilia : the official journal of the World Federation of Hemophilia Vol. 21; no. 4; pp. 496 - 501
Main Authors:	Santoro, C., Di Mauro, R., Baldacci, E., De Angelis, F., Abbruzzese, R., Barone, F., Bochicchio, R. A., Ferrara, G., Guarini, A., Foà, R., Mazzucconi, M. G.
Format:	Journal Article
Language:	English
Published:	England Blackwell Publishing Ltd 01-07-2015
Subjects:	Adolescent Adult Aged Aged, 80 and over Blood Loss, Surgical - prevention & control Cesarean Section Child Child, Preschool disease management factor XI Factor XI - therapeutic use factor XI deficiency Factor XI Deficiency - drug therapy Female haemorrhage Hemostasis, Surgical hereditary coagulation disorders Humans Infant Male Middle Aged Phenotype Postpartum Hemorrhage - prevention & control Pregnancy Retrospective Studies surgical procedures Young Adult surgical procedures haemorrhage factor XI factor XI deficiency hereditary coagulation disorders disease management
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Summary:	Summary Bleeding phenotype in factor XI (FXI)‐deficient patients is variable, and not related to baseline FXI:Act. Aims of our study were to describe the characteristics and the management of surgery and deliveries in FXI‐deficient patients, and to investigate the relationship between the haemorrhagic phenotype and the baseline FXI:Act. Ninety‐five patients were diagnosed and followed in our centre for a median follow‐up of 0.9 years (0.1–36.2); median FXI:Act of all patients: 38% (0.5–69%). Fifty‐six patients (59%) experienced bleeding episodes not surgery‐related. Prior to diagnosis, 64 patients underwent 132 surgeries, and after diagnosis, 23 patients underwent 36 surgeries. Globally 26 of 168 surgeries were prophylactically treated, whereas 142 of 168 were not. As regard as surgeries performed without prophylaxis, 30 bleeding events (21%) occurred in 21 patients. At diagnosis, the median FXI:Act of bleeding and non‐bleeding patients was 28% and 37%, respectively, without statistically significant difference between the two groups (P = 0.26). As regard as surgeries performed under prophylactic treatment just 1 bleeding event occurred. Prior to diagnosis, 31 spontaneous deliveries (SD) and eight caesarian sections (CS) were performed without prophylaxis: 4 postpartum haemorrhages (10.5%) occurred (patients FXI:Act: 2%, 6%, 27%, 52.3% respectively). After diagnosis, four SD and five CS were performed with prophylaxis: no postpartum haemorrhages occurred. We confirm the wide bleeding phenotype variability in FXI‐deficient patients, not related to the baseline FXI:Act levels. We highlight the importance of performing a correct diagnosis and follow‐up, because a good management of prophylactic treatment, dramatically reduces the bleeding rate in case of surgery or deliveries.
Bibliography:	istex:2BE75A6BF50865C0F17DEAA23D1EB50AD8208E58 ArticleID:HAE12628 ark:/67375/WNG-4DK93QTJ-5 ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	1351-8216 1365-2516
DOI:	10.1111/hae.12628