Hereditary coagulation factor XI deficiency : a rare or neglected disease? Results from a retrospective, single-centre cohort in northern Italy
Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved..
To examine real-life clinical data regarding hereditary factor XI (FXI) deficiency from a secondary care centre. Retrospective review of clinical records for every FXI:C 0.7 IU/ml or less reported from 2012 to 2020. Seventy-nine patients were included. Six (7.6%) had a severe deficiency (FXI:C <0.2 IU/ml). Only 55 (69.6%) patients were referred to the Haemostasis Centre. Among them, six (15%) were subsequently not identified at increased haemorrhagic risk before a surgical/obstetrical procedure. Thirty-three (41.8%) experienced at least one bleeding event, minor (25 patients) and/or major (16 patients). Minor bleedings were predominantly spontaneous and more frequent in women, major events were mainly provoked. No correlation was found between FXI:C and risk of bleeding ( P = 0.9153). Lower FXI:C, but not a positive bleeding history, was related with higher likelihood of being referred to the Haemostasis Centre ( P = 0.0333). Hereditary FXI deficiency prevalence is likely underestimated, real-life clinical practices outside reference centres could be suboptimal.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:35 |
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| Enthalten in: |
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis - 35(2024), 1 vom: 01. Jan., Seite 32-36 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Canovi, Simone [VerfasserIn] |
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| Anmerkungen: |
Date Completed 11.01.2024 Date Revised 01.02.2024 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1097/MBC.0000000000001270 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM36542594X |
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| 245 | 1 | 0 | |a Hereditary coagulation factor XI deficiency |b a rare or neglected disease? Results from a retrospective, single-centre cohort in northern Italy |
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| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved. | ||
| 520 | |a To examine real-life clinical data regarding hereditary factor XI (FXI) deficiency from a secondary care centre. Retrospective review of clinical records for every FXI:C 0.7 IU/ml or less reported from 2012 to 2020. Seventy-nine patients were included. Six (7.6%) had a severe deficiency (FXI:C <0.2 IU/ml). Only 55 (69.6%) patients were referred to the Haemostasis Centre. Among them, six (15%) were subsequently not identified at increased haemorrhagic risk before a surgical/obstetrical procedure. Thirty-three (41.8%) experienced at least one bleeding event, minor (25 patients) and/or major (16 patients). Minor bleedings were predominantly spontaneous and more frequent in women, major events were mainly provoked. No correlation was found between FXI:C and risk of bleeding ( P = 0.9153). Lower FXI:C, but not a positive bleeding history, was related with higher likelihood of being referred to the Haemostasis Centre ( P = 0.0333). Hereditary FXI deficiency prevalence is likely underestimated, real-life clinical practices outside reference centres could be suboptimal | ||
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| 700 | 1 | |a Leone, Maria Cristina |e verfasserin |4 aut | |
| 700 | 1 | |a Depietri, Luca |e verfasserin |4 aut | |
| 700 | 1 | |a Veropalumbo, Maria Rosaria |e verfasserin |4 aut | |
| 700 | 1 | |a Pilia, Annalisa |e verfasserin |4 aut | |
| 700 | 1 | |a Granito, Maria |e verfasserin |4 aut | |
| 700 | 1 | |a Bonanno, Antonio |e verfasserin |4 aut | |
| 700 | 1 | |a Casali, Annamaria |e verfasserin |4 aut | |
| 700 | 1 | |a Colla, Rossana |e verfasserin |4 aut | |
| 700 | 1 | |a Ghirarduzzi, Angelo |e verfasserin |4 aut | |
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