Several possible treatments are available for FXI deficiency.37 The pros and cons of each are shown in Table 1, and the treatment of specific clinical scenarios is considered in Table 2. These tables do not take into account the more recent research with thrombin generation tests (TGT), instead they base treatment choices on FXI:C levels and bleeding history. At the present time however, the TGT is not readily available.
Click on table to enlarge.
The first issue is to decide whether treatment is required. This decision will depend on the hemostatic challenge under consideration, the patient’s past medical history with interventions, if present, and to some extent on the level of deficiency.32 Many women with severe FXI deficiency have given birth without treatment aimed at increasing the FXI level; they would not be eligible for spinal anaesthesia in these circumstances.
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Rare Coagulation Disorders Resource Room, an extension of the RBDD Registry (eu.rbdd.org), was developed in collaboration with the Indiana Hemophilia & Thrombosis Center (www.innovativehematology.org).
The Resource Room serves as a platform to promote international collaboration to increase knowledge of these very rare disorders and to improve the care of affected individuals. The international RBDD mission, under the leadership of Dr. Peyvandi, will continue to be expanded over time.
The Resource Room thanks all contributing authors of the website articles that have made this valuable information available to the global community of both individuals affected with rare coagulation disorders and their healthcare providers.
