Acquired post partum hemophilia

We examine the international literature concerning acquired hemophilia subsequent to the observation of a 28-year-old woman who was brought to our attention for important metrorrhagia and hematuria which began on the 2nd day post-partum and led to 3 uterine cavity revisions in the following week, without any benefit. The hematochemical tests carried out on the patient presented the following picture: anemia (hemoglobin 5.9 g/dl, hematocrit 19%), normal platelet count and coagulation impairment: normal PT-INR, aPIT52.3 s (26-36 s), aPTT ratio 1.74 (0.8-1.2) ,factor (F) VIII 13% (60-150%), positive anti-FVIII inhibitors (usually absent with increasing title in the following days (0.70, 1.22 and 1.67 U/ml). A substitutive therapy was immediately begun with FVIII concentrates, at first as a bolus in order to allow a 4th cavity revision and then in continuous infusion as a preparation for laparohysterectomy. The dosage was gradually increased from 4U/kg/h (4,800 U/die) to 20 U/kg/h (24,000 U/die), according to the clinical and laboratory results. The infusion was continued for 10 days with progressive reductions with a minimal FVIII desired goal of 60%. In the meantime, corticosteroid therapy was begun, endovenously and then orally for a total of 23 consecutive days. At discharge (on day 40), the patient had been infused with 145000 FVIII and 11 concentrated red cell units; the FVIII dosage was 40% and no hemorrhagic manifestations were evident.