Treatment options in factor XI (FXI) deficiency.
| Treatment . | . | Advantages . | Disadvantages . |
|---|---|---|---|
| Watch and wait | Suitable for many individuals with partial deficiency | No exposure to blood products. | Hemorrhage may occur. |
| Fibrinolytic inhibitors | eg, Tranexamic acid | Effective as single agent for dental extractions even in severe deficiency. Good for menorrhagia. | Not available in some countries. |
| Fresh frozen plasma (FFP) | Standard FFP | Readily available. | Large volumes may be required Infection risk. |
| Pathogen-inactivated FFP | Safer than standard FFP. | Large volumes may be required. | |
| a) Solvent-detergent | Pooled donations. | ||
| b) Methylene blue | Single donor units. | Variable FXI activity. | |
| Factor XI concentrates | Factor XI BPL Hemoleven LFB | Predictable rise in FXI level, small volume of infusion. | Not licensed or available in many countries. Thrombotic risk especially in those with predisposition. |
| rVIIa | Small volume. | Not licensed for this indication. | |
| Instant action. Effective for patients with inhibitors. | Thrombotic risk, the safe and effective dose yet to be decided. | ||
| Desmopressin | Non-blood product. Easy to administer. | No clear evidence of benefit in reported series. Need for fluid restriction for 24 h. |
| Treatment . | . | Advantages . | Disadvantages . |
|---|---|---|---|
| Watch and wait | Suitable for many individuals with partial deficiency | No exposure to blood products. | Hemorrhage may occur. |
| Fibrinolytic inhibitors | eg, Tranexamic acid | Effective as single agent for dental extractions even in severe deficiency. Good for menorrhagia. | Not available in some countries. |
| Fresh frozen plasma (FFP) | Standard FFP | Readily available. | Large volumes may be required Infection risk. |
| Pathogen-inactivated FFP | Safer than standard FFP. | Large volumes may be required. | |
| a) Solvent-detergent | Pooled donations. | ||
| b) Methylene blue | Single donor units. | Variable FXI activity. | |
| Factor XI concentrates | Factor XI BPL Hemoleven LFB | Predictable rise in FXI level, small volume of infusion. | Not licensed or available in many countries. Thrombotic risk especially in those with predisposition. |
| rVIIa | Small volume. | Not licensed for this indication. | |
| Instant action. Effective for patients with inhibitors. | Thrombotic risk, the safe and effective dose yet to be decided. | ||
| Desmopressin | Non-blood product. Easy to administer. | No clear evidence of benefit in reported series. Need for fluid restriction for 24 h. |