Treatment options for acquired hemophilia A
| Treatment . | Dosing and clinical recommendations . |
|---|---|
| Treatment of acute bleeding | |
| Bypassing agents | |
| aPCC | 50-100 IU/kg iv every 8-12 hours until clinical response |
| rFVIIa | 90-120 μg/kg iv every 2-3 hours until clinical response |
| Treatments to raise circulating FVIII levels | |
| Porcine FVIII concentrates | Not currently available for routine clinical use |
| Human FVIII concentrates | Patients with inhibitor titer < 5 BU: 20 IU/kg iv for each BU of inhibitor plus 40 IU/Kg iv |
| Desmopressin | Patients with inhibitor titer < 5BU and minor bleeding episodes: 0.3 μg/kg iv/sc |
| Inhibitor eradication | |
| Immunosuppressive agents | |
| Prednisone plus cyclophosphamide | Prednisone (1 mg/kg per day) plus cyclophosphamide (1-2 mg/kg per day) po for at least 5 weeks |
| Cyclosporine | 200-300 mg/day alone or in association with prednisone as second-line therapy |
| High-dose intravenous immunoglobulin | 0.4 g/kg per day for 5 days or 1.0 g/kg per day for 2 days in association with other treatments (steroids, immunoadsorption, IT regimens) |
| Immunoadsorption | Rapid but transitory removal of the inhibitor; in association with FVIII concentrates or IT |
| Immune tolerance | FVIII concentrates in combination with various eradication therapies (DiMichele33 ; Green34 ; and Nemes and Pitlik35 ) |
| Rituximab | 375 mg/m2 weekly for 4 weeks as second-line therapy in association with steroids |
| Treatment . | Dosing and clinical recommendations . |
|---|---|
| Treatment of acute bleeding | |
| Bypassing agents | |
| aPCC | 50-100 IU/kg iv every 8-12 hours until clinical response |
| rFVIIa | 90-120 μg/kg iv every 2-3 hours until clinical response |
| Treatments to raise circulating FVIII levels | |
| Porcine FVIII concentrates | Not currently available for routine clinical use |
| Human FVIII concentrates | Patients with inhibitor titer < 5 BU: 20 IU/kg iv for each BU of inhibitor plus 40 IU/Kg iv |
| Desmopressin | Patients with inhibitor titer < 5BU and minor bleeding episodes: 0.3 μg/kg iv/sc |
| Inhibitor eradication | |
| Immunosuppressive agents | |
| Prednisone plus cyclophosphamide | Prednisone (1 mg/kg per day) plus cyclophosphamide (1-2 mg/kg per day) po for at least 5 weeks |
| Cyclosporine | 200-300 mg/day alone or in association with prednisone as second-line therapy |
| High-dose intravenous immunoglobulin | 0.4 g/kg per day for 5 days or 1.0 g/kg per day for 2 days in association with other treatments (steroids, immunoadsorption, IT regimens) |
| Immunoadsorption | Rapid but transitory removal of the inhibitor; in association with FVIII concentrates or IT |
| Immune tolerance | FVIII concentrates in combination with various eradication therapies (DiMichele33 ; Green34 ; and Nemes and Pitlik35 ) |
| Rituximab | 375 mg/m2 weekly for 4 weeks as second-line therapy in association with steroids |
aPCC indicates activated prothrombin complex concentrates; rFVIIa, recombinant activated factor VII; iv, intravenously; sc, subcutaneously; IT, immune tolerance; and po, by mouth.