Clinical management of different scenarios in factor XI (FXI) deficiency.
| Clinical scenario . | Management of severe deficiency . | Management of partial deficiency . | Cautions and comments . |
|---|---|---|---|
| Notes: Assuming that other causes of bleeding have been investigated and excluded (eg, von Willebrand disease, platelet disorders). All patients should avoid aspirin or other non-steroidal anti-inflammatory agents within the 7 days prior to and following surgery. | |||
| *Replacement therapy means either FXI concentrate or FFP with the cautions noted in Table 1. | |||
| Circumcision | If circumcision is required for religious reasons in the first few days of life, male children at risk should have a level done at birth. Bleeding is uncommon (1.5%) and Israeli experience indicates that replacement is not mandatory.32 An alternative is to delay surgery or to use replacement therapy.*42 | No indication for replacement therapy.* | The level at birth is lower than the adult level, so children with apparent partial deficiency should be retested at >6 months of age to establish true level. |
| Labor and delivery39,40 | Many women with severe deficiency experience no excessive bleeding. Oral antifibrinolytic agents are advisable to reduce blood loss post-partum. There is an increased risk of hemorrhage if treatment to raise FXI level is not used. Spinal and epidural anesthesia is contraindicated unless FXI level is corrected to normal (and documented). | Expectant management unless previous bleeding history. In the absence of a bleeding history with adequate challenge, spinal anesthesia may be considered. Oral antifibrinolytics will reduce postpartum bleeding and may be useful for 2 wks. | Neonatal bleeding is very rare even in severe deficiency, but obstetricians should manage delivery of the ‘at risk’ infant with caution, avoiding instrumentation as far as possible, and the FXI level measured from cord blood in males who may require circumcision. |
| Major surgery in areas of high fibrinolytic activity, including tonsillectomy or prostatectomy | Replacement therapy* to achieve trough levels of 45 IU/dL for 5 to 7 days together with oral antifibrinolytic agents. Regular monitoring of FXI level is required to determine dosing. | Replacement therapy* for those with previous bleeding history together with antifibrinolytic agents. | |
| Major surgery not associated with increased fibrinolysis, eg, orthopedic surgery, appendectomy | Replacement therapy* usually required as above, but even without this bleeding is uncommon.32 | Expectant management with close observation and attention to hemostasis. | |
| Minor surgery including skin biopsies | No replacement therapy.* | No replacement therapy.* | |
| Dental extractions | Oral antifibrinolytic agent alone starting the night before and continuing for 7 days. | If previous bleeding history as for severe deficiency. If no bleeding history despite challenge, expectant management only. | |
| Menorrhagia | Oral antifibrinolytic agents for the days with heaviest bleeding; if unsuccessful move to hormonal control with oral contraceptives. | As for severe deficiency. | |
| Surgical management of patients with FXI inhibitors33 | rVIIa is effective at low dose of 15 μg/kg for major surgery used with oral tranexamic acid. | Inhibitors not described in partial deficiency. | Higher doses of rVIIa (90 μg/kg have been associated with increased thrombotic risk |
| Clinical scenario . | Management of severe deficiency . | Management of partial deficiency . | Cautions and comments . |
|---|---|---|---|
| Notes: Assuming that other causes of bleeding have been investigated and excluded (eg, von Willebrand disease, platelet disorders). All patients should avoid aspirin or other non-steroidal anti-inflammatory agents within the 7 days prior to and following surgery. | |||
| *Replacement therapy means either FXI concentrate or FFP with the cautions noted in Table 1. | |||
| Circumcision | If circumcision is required for religious reasons in the first few days of life, male children at risk should have a level done at birth. Bleeding is uncommon (1.5%) and Israeli experience indicates that replacement is not mandatory.32 An alternative is to delay surgery or to use replacement therapy.*42 | No indication for replacement therapy.* | The level at birth is lower than the adult level, so children with apparent partial deficiency should be retested at >6 months of age to establish true level. |
| Labor and delivery39,40 | Many women with severe deficiency experience no excessive bleeding. Oral antifibrinolytic agents are advisable to reduce blood loss post-partum. There is an increased risk of hemorrhage if treatment to raise FXI level is not used. Spinal and epidural anesthesia is contraindicated unless FXI level is corrected to normal (and documented). | Expectant management unless previous bleeding history. In the absence of a bleeding history with adequate challenge, spinal anesthesia may be considered. Oral antifibrinolytics will reduce postpartum bleeding and may be useful for 2 wks. | Neonatal bleeding is very rare even in severe deficiency, but obstetricians should manage delivery of the ‘at risk’ infant with caution, avoiding instrumentation as far as possible, and the FXI level measured from cord blood in males who may require circumcision. |
| Major surgery in areas of high fibrinolytic activity, including tonsillectomy or prostatectomy | Replacement therapy* to achieve trough levels of 45 IU/dL for 5 to 7 days together with oral antifibrinolytic agents. Regular monitoring of FXI level is required to determine dosing. | Replacement therapy* for those with previous bleeding history together with antifibrinolytic agents. | |
| Major surgery not associated with increased fibrinolysis, eg, orthopedic surgery, appendectomy | Replacement therapy* usually required as above, but even without this bleeding is uncommon.32 | Expectant management with close observation and attention to hemostasis. | |
| Minor surgery including skin biopsies | No replacement therapy.* | No replacement therapy.* | |
| Dental extractions | Oral antifibrinolytic agent alone starting the night before and continuing for 7 days. | If previous bleeding history as for severe deficiency. If no bleeding history despite challenge, expectant management only. | |
| Menorrhagia | Oral antifibrinolytic agents for the days with heaviest bleeding; if unsuccessful move to hormonal control with oral contraceptives. | As for severe deficiency. | |
| Surgical management of patients with FXI inhibitors33 | rVIIa is effective at low dose of 15 μg/kg for major surgery used with oral tranexamic acid. | Inhibitors not described in partial deficiency. | Higher doses of rVIIa (90 μg/kg have been associated with increased thrombotic risk |