Types of hemostatic agents for hemophilia
| Hemophilia A without high titer inhibitor . | Hemophilia A with high titer inhibitor (>5 BU) . | Hemophilia B–specific agents . | Hemophilia B with inhibitor . | Adjuvant agents . |
|---|---|---|---|---|
| Standard half-life FVIII CFCs | aPCCs, ie, FVIII bypassing agents (FEIBA)c | Standard half-life FIX CFCs | Activated rFVIIad | Antifibrinolytice |
| Extended half-life FVIII CFCs | Activated rFVIIad | Extended half-life FIX CFCs | Topical fibrin glue | |
| Desmopressina | Emicizumabb | Transfusion support | ||
| Emcizumabb |
| Hemophilia A without high titer inhibitor . | Hemophilia A with high titer inhibitor (>5 BU) . | Hemophilia B–specific agents . | Hemophilia B with inhibitor . | Adjuvant agents . |
|---|---|---|---|---|
| Standard half-life FVIII CFCs | aPCCs, ie, FVIII bypassing agents (FEIBA)c | Standard half-life FIX CFCs | Activated rFVIIad | Antifibrinolytice |
| Extended half-life FVIII CFCs | Activated rFVIIad | Extended half-life FIX CFCs | Topical fibrin glue | |
| Desmopressina | Emicizumabb | Transfusion support | ||
| Emcizumabb |
Desmopressin may be used only for minor bleeding for some patients with mild hemophilia A; desmopressin has an expected increase in FVIII of 2-4-fold and can be used 30 to 60 minutes prior to the procedure. Due to the risk of tachyphylaxis after 1 to 2 doses, desmopressin should not be used if patients need increased FVIII levels for 2 to 3 days postop.11
Emicizumab is used for hemostatic prophylaxis. Please see details in the section on emicizumab for additional information.
The WFH recommends against the use of aPCC/FEIBA for patients with congenital hemophilia B and inhibitors due to the risk of accumulation of clotting factors II, VII, and X, which can be associated with a higher risk of thrombotic complications.
rFVIIa is preferred for patients with hemophilia A with inhibitors on emicizumab, as well as patients with hemophilia B with a history of anaphylaxis to FIX products, which can be indicative of an underlying inhibitor to FIX.
TXA and epsilon-aminocaproic acid should be avoided in patients with a history of an acute thrombosis, disseminated intravascular coagulation, or thrombotic microangiopathy.