Summary of patients experiencing vascular accident
| Patient no. . | Factor VIII level . | Mutation . | Inhibitor status prior to thrombosis . | Catheter used . | Duration of catheter prior to thrombosis . | Factor concentrate . | Mode of application prior to vascular event . | Prothrombotic risk factor . | Exogenous trigger . |
|---|---|---|---|---|---|---|---|---|---|
| 1 | < 1% | Intron 22 | High responder | Port | 1.5 years | rFVIII | On demand | FV 1691G>A | None |
| 2 | < 1% | 2781delT (exon 14) | High responder | Port | 1.8 years | pdFVIII | Prophylaxis 3/wk | Lp(a)* | None |
| 3 | < 1% | ND | — | Hickman | 4 months | FVIII/vWF | Continuous infusion: bleeding episode | ND | None |
| 4 | 1.4% | ND | — | Port | 1.5 years | FVIII/vWF | Prophylaxis 3/wk | MTHFR TT | Obesity |
| 5 | < 1% | Intron 22 | — | Port | 7 years | rFVIII | Prophylaxis 3/wk | FV 1691G>A | None |
| 6 | < 1%† | Intron 22 | — | Port | 6 years | FVIII/vWF | Prophylaxis 3/wk | MTHFR TT | None |
| 7 | < 1%† | Intron 22 | Low responder | Port | 10 years | pdFVIII | IT following prophylaxis 3/wk | MTHFR TT | None |
| 8 | < 1% | Intron 22 | High responder | Port | 7 months | FVIII/vWF | Prophylaxis 3/wk | No risk identified so far | None |
| Patient no. . | Factor VIII level . | Mutation . | Inhibitor status prior to thrombosis . | Catheter used . | Duration of catheter prior to thrombosis . | Factor concentrate . | Mode of application prior to vascular event . | Prothrombotic risk factor . | Exogenous trigger . |
|---|---|---|---|---|---|---|---|---|---|
| 1 | < 1% | Intron 22 | High responder | Port | 1.5 years | rFVIII | On demand | FV 1691G>A | None |
| 2 | < 1% | 2781delT (exon 14) | High responder | Port | 1.8 years | pdFVIII | Prophylaxis 3/wk | Lp(a)* | None |
| 3 | < 1% | ND | — | Hickman | 4 months | FVIII/vWF | Continuous infusion: bleeding episode | ND | None |
| 4 | 1.4% | ND | — | Port | 1.5 years | FVIII/vWF | Prophylaxis 3/wk | MTHFR TT | Obesity |
| 5 | < 1% | Intron 22 | — | Port | 7 years | rFVIII | Prophylaxis 3/wk | FV 1691G>A | None |
| 6 | < 1%† | Intron 22 | — | Port | 6 years | FVIII/vWF | Prophylaxis 3/wk | MTHFR TT | None |
| 7 | < 1%† | Intron 22 | Low responder | Port | 10 years | pdFVIII | IT following prophylaxis 3/wk | MTHFR TT | None |
| 8 | < 1% | Intron 22 | High responder | Port | 7 months | FVIII/vWF | Prophylaxis 3/wk | No risk identified so far | None |
All 8 patients have hemophilia A.
F indicates factor; IT, immune tolerance therapy; Lp(a), lipoprotein (a); MTHFR TT, thermolabile methylenetetrahydrofolate reductase 677C>T genotype; ND, not determined; pdFVIII, purified factor VIII concentrate; rFVIII, recombinant factor VIII concentrate; FVIII/vWF, factor VIII/von Willebrand factor concentrate.
Above 30 mg/dL.
Catheter welded together with vessel wall and could not be completely removed.