TABLE 4

Summary of patients with acquired hemophilia A and thrombosis

StudyRisk factors for thrombosisAt diagnosisHematologic treatmentImmunosuppression regimen
Activated partial thromboplastin time, secondsFactor VIII, U/dLInhibitor, Bethesda units/mL
1. Poli et al18Pregnancy57 at deep vein thrombosis diagnosis and on anticoagulation1.9NAVitamin KPrednisone 1 g/kg/day (4 weeks)
2. Deitcher et al19Idiopathic54.3 (24–33)5.357Factor VIII concentrate and desmopressinNA
Lymphoma61.4 (22–33)247Factor VIII concentrate and desmopressinNA
Postoperative54.8 (22–33)25Factor VIII concentrate and desmopressinNA
3. Spencer et al20Pregnancy3.01235Recombinant activated factor VIIPrednisolone 1 mg/kg/day (6 weeks) and cyclophosphamide 100 mg/day (6 months)
4. Paudel et al21Trauma or surgery> 100< 112Vitamin K, fresh frozen plasma, recombinant activated factor VIIMethylprednisolone 120 mg/day, then rituximab 375 mg/m2/weekly for 4 doses
5. Wool et al22Idiopathic76.9 (24–34)427Factor VIII concentrate (2 days), then recombinant activated factor VII (30 μg/kg/dose), then therapeutic plasma exchangeDexamethasone 8 mg/12 hours, therapeutic plasma exchange days 5 and 6, then rituximab 375 mg/m2/week (2 doses)
6. Maral et al23Malignancy107 (22–34)3350NAPrednisolone 1 mg/kg/day and cyclophosphamide 500 mg/week
7. Chhabra et al24Idiopathic78.6< 182Recombinant activated factor VIIPrednisolone 1 mg/kg/day and cyclophosphamide 100 mg/day
8. Aslam et al25Addison disease81.8< 6NAActivated prothrombin complex concentrate, vitamin KIntravenous methylprednisolone 30 mg/12 hours
  • NA = not available