TREATMENT OF BLEEDING AND FACTOR VIII INHIBITORS IN ACQUIRED HEMOPHILIA A PATIENTS
Abstract
Background. Bleeding in acquired hemophilia A patients with FVIII autoantibodies is often severe. The authors describe a successfuly acquired hemophilia A treatment with Feiba, glucocorticoids and cyclophosphamide in three patients.
Results. In a 66-year patient with chest hematoma and m. iliopsoas hematoma, APTT 50.1 s, FVIII 0.05 IU/mL and inhibitors FVIII (anti-FVIII) 22 BU/ml were found. After treatment of hemorrhages with Feiba, dexamethasone and cyclophosphamide, FVIII increased to 0.54 IU/mL and no inhibitors were found on day seventeen. In a 46-year patient with advanced AIDS, multiple hematomas and liver cirrhosis after HCV infection, the platelet count was 92 × 109/L, APTT 48.2 s, FVIII 0.05 IU/mL and anti-FVIII 1.7 BU/mL. The hemorrhages were treated with FEIBA and methylprednisolone therapy was started after 14 months. After 42 days FVIII increased to 0.56 IU/mL and no inhibitors were found. In a 69-year patient suffering from thyroiditis and idiopathic thrombocytopenic purpura, extensive gluteal and chest hematomas were detected during the maintenance therapy with methylprednisolone 16 mg/day. The thrombocyte count was normal but APTT 48.8 s, FVIII 0.01 IU/mL and anti-FVIII 7.0 BU/mL was found. FVIII activity were 1.72 IU/mL and no inhibitors was noted after 330-days of treatment with cyclophosphamide.
Conclusions. The treatment with FEIBA was effective in stopping the hemorrhages. The glucocorticoids were efficient in prompt decreasing of the inhibitor titer whereas the effect of cyclophosphamide was retarded.
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