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Uptodate Reference Title
Management of delivery in a pregnant woman with factor XI deficiency and factor XI activity level ≤40%
Management of delivery in a pregnant woman with factor XI deficiency and factor XI activity level ≤40%
This algorithm only applies to individuals with known factor XI deficiency and factor XI activity ≤40% with an otherwise uncomplicated pregnancy (no major bleeding or thrombotic complications). It does not take the place of the judgment of the clinician who knows the patient's medical history and values and preferences, or the advice of the local hemophilia treatment center. Most asymptomatic individuals with factor XI levels >40% can be managed similarly to those without factor XI deficiency. All others should have a delivery plan developed by a multidisciplinary team. If neuraxial anesthesia is considered, early consultation with the anesthesiology team is advised (typically in the third trimester, before labor has commenced) to determine the factor XI level at which neuraxial procedures will be performed.
* A negative bleeding history refers to absence of bleeding despite true bleeding challenges such as surgery, prior childbirth, or invasive procedures. Lack of bleeding alone is insufficient to state that the bleeding history is negative. ¶ Prophylaxis is usually done with factor XI replacement during the first stage of labor or before cesarean delivery, using a factor XI concentrate (available in Canada [under a special access program] and Europe) or a plasma product such as Fresh Frozen Plasma (FFP).
If plasma is used in a woman with a very low factor XI level, elective (scheduled) delivery may be appropriate to allow time for transfusion.
A typical target for factor XI activity is 20 to 40%. An alternative is low-dose rFVIIa (eg, 15 to 30 mg/kg); this may be used in individuals with severe factor XI deficiency with an inhibitor, in those in whom inhibitor development is a concern, or in those who place a strong value on avoiding exposure to plasma.
Plasma or low-dose rFVIIa may be used concurrently with antifibrinolytic therapy.
Caution is used when combining a factor XI concentrate with antifibrinolytic therapy. Refer to UpToDate for details.
Δ All women should be considered for intravenous tranexamic acid (dose, 1 gram) at the time of neuraxial anesthesia or deliver. Prophylactic antifibrinolytic therapy is typically continued for 3 days after vaginal delivery or 5 days after cesarean delivery. Extended prophylaxis may not be needed in women with mild deficiency (factor XI level >20%) and a negative bleeding history. If bleeding occurs despite antifibrinolytic therapy, factor XI replacement may be required. Antifibrinolytic agents are generally avoided if hematuria is present.
