| | Summary | Interventions |
| Bleeding risk in FXI deficiency | - Bleeding, if it occurs, is typically in the setting of surgery or trauma.
- There may be heavy menstrual bleeding (HMB), but spontaneous bleeding is uncommon.
- Correlations of F11 genotype, bleeding risk, and FXI activity are variable.
- Normal FXI activity excludes FXI deficiency.
| - Obtain bleeding history.
- Laboratory testing:
- PT
- aPTT
- Mixing study as indicated
- FXI activity level
- Review case with a hemophilia treatment center (HTC) or other center of excellence.
|
| Patient education | - Review risks of bleeding and inhibitor development.
- Genetic counseling.
- Preconception testing and counseling as appropriate.
- Counseling and testing first-degree relatives as appropriate.
- Possible interventions for HMB.
- Planning for surgery and/or pregnancy.
| - Medical alert bracelet and/or wallet card.
- Avoid antiplatelet agents for routine pain or fever.
- Inhibitor testing for selected individuals.
- HMB can be treated with an antifibrinolytic agent during the first few days of the menstrual cycle or with hormonal suppressive therapy.
- Consult with the HTC if antiplatelet agent or anticoagulant is needed for another indication.
|
| Surgery/invasive procedures | - Various therapies are available to reduce bleeding risk or treat bleeding.*
- Therapies may be administered prophylactically or held in reserve for bleeding.
- Modify as appropriate (surgery, anesthesia, or site of care).
| - Discuss with the HTC or center of excellence with time for advance planning.
|
| Pregnancy | - Variable bleeding during pregnancy.
- Increased risk of postpartum hemorrhage.
| - Prenatal counseling and testing as appropriate; paternal testing may be indicated.
- Manage in consultation with the HTC or center of excellence.
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