Summary of patients with acquired hemophilia A and thrombosis
| Study | Risk factors for thrombosis | At diagnosis | Hematologic treatment | Immunosuppression regimen | ||
|---|---|---|---|---|---|---|
| Activated partial thromboplastin time, seconds | Factor VIII, U/dL | Inhibitor, Bethesda units/mL | ||||
| 1. Poli et al18 | Pregnancy | 57 at deep vein thrombosis diagnosis and on anticoagulation | 1.9 | NA | Vitamin K | Prednisone 1 g/kg/day (4 weeks) | 
| 2. Deitcher et al19 | Idiopathic | 54.3 (24–33) | 5.3 | 57 | Factor VIII concentrate and desmopressin | NA | 
| Lymphoma | 61.4 (22–33) | 2 | 47 | Factor VIII concentrate and desmopressin | NA | |
| Postoperative | 54.8 (22–33) | 2 | 5 | Factor VIII concentrate and desmopressin | NA | |
| 3. Spencer et al20 | Pregnancy | 3.01 | 2 | 35 | Recombinant activated factor VII | Prednisolone 1 mg/kg/day (6 weeks) and cyclophosphamide 100 mg/day (6 months) | 
| 4. Paudel et al21 | Trauma or surgery | > 100 | < 1 | 12 | Vitamin K, fresh frozen plasma, recombinant activated factor VII | Methylprednisolone 120 mg/day, then rituximab 375 mg/m2/weekly for 4 doses | 
| 5. Wool et al22 | Idiopathic | 76.9 (24–34) | 4 | 27 | Factor VIII concentrate (2 days), then recombinant activated factor VII (30 μg/kg/dose), then therapeutic plasma exchange | Dexamethasone 8 mg/12 hours, therapeutic plasma exchange days 5 and 6, then rituximab 375 mg/m2/week (2 doses) | 
| 6. Maral et al23 | Malignancy | 107 (22–34) | 3 | 350 | NA | Prednisolone 1 mg/kg/day and cyclophosphamide 500 mg/week | 
| 7. Chhabra et al24 | Idiopathic | 78.6 | < 1 | 82 | Recombinant activated factor VII | Prednisolone 1 mg/kg/day and cyclophosphamide 100 mg/day | 
| 8. Aslam et al25 | Addison disease | 81.8 | < 6 | NA | Activated prothrombin complex concentrate, vitamin K | Intravenous methylprednisolone 30 mg/12 hours | 
NA = not available