English
Admission may be required if bleeding is not readily controlled with outpatient or emergency department care; length of stay for invasive procedures (or childbirth) may be increased
Prophylaxis (periprocedural) and treatment include replacement of factor XI and/or use of antifibrinolytic drugs (ie, tranexamic acid, aminocaproic acid) r5
Determining the need for prophylaxis is problematic r8
There are few published guidelines with specific recommendations for management of hemophilia C; UK Haemophilia Centre Doctors' Organisation offers the following: r11r17
Clinical situation | Appropriate treatment |
---|---|
Minor bleeds or minor surgery in patients with higher bleeding risk | Tranexamic acid |
All bleeds and all surgery in patients with lower bleeding risk | Tranexamic acid |
Severe bleeding or major surgery in patients with higher bleeding risk | A dose of factor XI concentrate (if available), or a combination of fresh frozen plasma and tranexamic acid |
Labor and delivery of women with recent factor XI level of 15 to 70 units/dL and a history of excessive bleeding or no relevant hemostatic challenge | Tranexamic acid |
Labor and delivery of women with recent factor XI level of less than 15 units/dL and a bleeding history or an unknown bleeding history | A dose of factor XI concentrate (if available), fresh frozen plasma, or rFVIIa |
Labor and delivery of women with recent factor XI level of 15 to 70 units/dL and a history of relevant hemostatic challenge without excessive bleeding | Manage expectantly; consider tranexamic acid in women with history of excessive bleeding |
Menorrhagia can be controlled with antifibrinolytic agents; an intrauterine device or oral contraceptives may be considered r4
World Federation of Hemophilia recommends immunization against hepatitis A and B for all patients with hemophilia r18