Summary of FXI-deficient patients treated with anticoagulant therapy (N = 15)
| ID . | Sex . | Age (y) . | Mutation (status) . | FXI:C (%) . | Bleeding history pre-AC . | Indication . | Drug . | Dose . | Therapy length (mo) . | For VKA, TTR (%) . | Other antithrombotics . | Bleeding post-AC (WHO grade) . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | 68 | p.P538L (+/+) | 23 | No | AF, NSTEMI | Acenocoumarol | 10.5 mg/wk | 2.5 | 66 | ASA & Clopidogrel | No |
| 2 | F | 70 | p.C56R (+/−) | 50 | No | AF, By-pass | Acenocoumarol | 11 mg/wk | 10.9 | 92 | Clopidogrel | No |
| 3 | M | 61 | p.C56R (+/−) | 66 | No | AF | Rivaroxaban | 20 mg qd | 2.3 | NA | No | No |
| 4 | M | 85 | p.C416Y (+/−) | 24 | UGB (ulcer), rectal bleeding | AF, TIA | Acenocoumarol | 4.75 mg/wk | 58.0 | 75 | No | Rectal bleeding (grade 1) |
| 5 | M | 80 | p.C599Y (+/−) | 47 | No | AF | Acenocoumarol | 11 mg/wk | 35.7 | 48 | No | No |
| Apixaban | 5 mg bd | 13.6 | NA | No | No | |||||||
| 6 | F | 92 | p. I426T | 39 | No | AF | Acenocoumarol | 8.5 mg/wk | 33.0 | 65 | No | No |
| 7 | F | 74 | p.C416Y (+/−) | 71 | No | AF, stroke | Acenocoumarol | 21 mg/wk | 235.5 | 80 | No | No |
| 8 | M | 34 | p.C56R (+/−) | 31 | No | DVT | Acenocoumarol | 6 mg/wk | 4.0 | 83 | LMWH (1 wk before oral AC) | No |
| 9 | F | 73 | p.C56R (+/−) | 25 | No | AF | Acenocoumarol | 5 mg/wk | 11.4 | 19 | No | No |
| Edoxaban | 60 mg qd | 10.6 | NA | No | No | |||||||
| 10 | M | 69 | p.C416Y (+/−) | 41 | No | AF | Acenocoumarol | 31.5 mg/wk | 124.2 | 61 | No | No |
| Edoxaban | 60 mg qd | 6.8 | NA | No | Gingiva bleeding (grade 1) | |||||||
| 11 | F | 26 | Ins 1653 bp (+/−) | 46 | No | Univentricular surgery; TGA & PS | Acenocoumarol | 14 mg/wk | 31 | 55 | ASA | No |
| Post stent | 26 mg/wk | 200 | 60 | ASA | No | |||||||
| 12 | F | 70 | p.R268C (+/−) | 35 | No | AF | Acenocoumarol | 10 mg/wk | 75.7 | 55 | No | No |
| 13 | M | 80 | ND | 40 | Tooth extraction | AF | Acenocoumarol | 16 mg/wk | 90.0 | 100 | No | No |
| 14 | F | 78 | p.C416Y | 38 | No | AF | Acenocoumarol | 12 mg/wk | 30.0 | 81 | LMWH (2 mo before oral AC) | No |
| 15 | M | 52 | p.E135* (+/−) | 59 | No | NSTEMI (aPLS) | Acenocoumarol | 8 mg/wk | 45.0 | 74 | No | No |
| ID . | Sex . | Age (y) . | Mutation (status) . | FXI:C (%) . | Bleeding history pre-AC . | Indication . | Drug . | Dose . | Therapy length (mo) . | For VKA, TTR (%) . | Other antithrombotics . | Bleeding post-AC (WHO grade) . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | 68 | p.P538L (+/+) | 23 | No | AF, NSTEMI | Acenocoumarol | 10.5 mg/wk | 2.5 | 66 | ASA & Clopidogrel | No |
| 2 | F | 70 | p.C56R (+/−) | 50 | No | AF, By-pass | Acenocoumarol | 11 mg/wk | 10.9 | 92 | Clopidogrel | No |
| 3 | M | 61 | p.C56R (+/−) | 66 | No | AF | Rivaroxaban | 20 mg qd | 2.3 | NA | No | No |
| 4 | M | 85 | p.C416Y (+/−) | 24 | UGB (ulcer), rectal bleeding | AF, TIA | Acenocoumarol | 4.75 mg/wk | 58.0 | 75 | No | Rectal bleeding (grade 1) |
| 5 | M | 80 | p.C599Y (+/−) | 47 | No | AF | Acenocoumarol | 11 mg/wk | 35.7 | 48 | No | No |
| Apixaban | 5 mg bd | 13.6 | NA | No | No | |||||||
| 6 | F | 92 | p. I426T | 39 | No | AF | Acenocoumarol | 8.5 mg/wk | 33.0 | 65 | No | No |
| 7 | F | 74 | p.C416Y (+/−) | 71 | No | AF, stroke | Acenocoumarol | 21 mg/wk | 235.5 | 80 | No | No |
| 8 | M | 34 | p.C56R (+/−) | 31 | No | DVT | Acenocoumarol | 6 mg/wk | 4.0 | 83 | LMWH (1 wk before oral AC) | No |
| 9 | F | 73 | p.C56R (+/−) | 25 | No | AF | Acenocoumarol | 5 mg/wk | 11.4 | 19 | No | No |
| Edoxaban | 60 mg qd | 10.6 | NA | No | No | |||||||
| 10 | M | 69 | p.C416Y (+/−) | 41 | No | AF | Acenocoumarol | 31.5 mg/wk | 124.2 | 61 | No | No |
| Edoxaban | 60 mg qd | 6.8 | NA | No | Gingiva bleeding (grade 1) | |||||||
| 11 | F | 26 | Ins 1653 bp (+/−) | 46 | No | Univentricular surgery; TGA & PS | Acenocoumarol | 14 mg/wk | 31 | 55 | ASA | No |
| Post stent | 26 mg/wk | 200 | 60 | ASA | No | |||||||
| 12 | F | 70 | p.R268C (+/−) | 35 | No | AF | Acenocoumarol | 10 mg/wk | 75.7 | 55 | No | No |
| 13 | M | 80 | ND | 40 | Tooth extraction | AF | Acenocoumarol | 16 mg/wk | 90.0 | 100 | No | No |
| 14 | F | 78 | p.C416Y | 38 | No | AF | Acenocoumarol | 12 mg/wk | 30.0 | 81 | LMWH (2 mo before oral AC) | No |
| 15 | M | 52 | p.E135* (+/−) | 59 | No | NSTEMI (aPLS) | Acenocoumarol | 8 mg/wk | 45.0 | 74 | No | No |
AC, anticoagulation; AF, atrial fibrillation; aPLS, antiphospholipid syndrome; ASA, acetylsalicylic acid; bd, twice daily; DVT, deep venous thrombosis; F, female; LMWH, low-molecular-weight heparin; M, male; NA, not applicable; NSTEMI, non-ST segment elevation myocardial infarction; PS, pulmonary stenosis; qd, every day; TGA, transposition of great arteries; TIA, transient ischemic attack; UGB, upper gastrointestinal bleeding.