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