Delivery and postpartum management in individuals with factor XI deficiency

All individuals with factor XI deficiency should have factor XI activity level measured during the third trimester. Those with factor XI activity >40% can be managed by routine obstetric care. Those with factor XI activity ≤40% and/or positive bleeding history should be managed by multidisciplinary care. If neuraxial anesthesia is considered, consult the anesthesia team early (typically in third trimester before labor has commenced) to discuss the safety of neuraxial anesthesia and other options. Factor XI replacement is typically not administered solely for the purpose of neuraxial anesthesia. When given prior to delivery, factor XI should generally be administered during the first stage of labor or before cesarean delivery. Plasma (eg, FFP) or rFVIIa can be used concomitantly with TXA; refer to UpToDate for dosing. Use caution when combining a factor XI concentrate with TXA; refer to UpToDate for details.

TXA: tranexamic acid; rFVIIa: recombinant activated factor VII; FFP: Fresh Frozen Plasma.

* Factor XI replacement can be provided by factor XI concentrate (not available in all jurisdictions) or a plasma product such as FFP; the choice between them is individualized (refer to UpToDate for dosing and other details).

¶ A typical dose of intravenous TXA is 1 gram. TXA dosing may be repeated; refer to UpToDate and product labeling for dosing. Postpartum, the duration of oral TXA may be extended beyond 5 days if excessive bleeding continues after ruling out obstetric causes of bleeding.

Δ Most individuals with factor XI >40% can be managed with routine obstetric care, including neuraxial anesthesia if desired. For the rare individual with a positive bleeding history, a delivery plan with a multidisciplinary team should be in place.

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Delivery and postpartum management in individuals with factor XI deficiency