Despite anti-haemorrhagic therapy with proper doses of activated prothrombin complex concentrate (aPCC, Feiba®), patients with acquired haemophilia A (AHA) have a considerable risk of recurrent bleeding complications. Evidence in support of the benefit-to-risk ratio of prevention strategies with the use of lower doses of aPCC following the end of the initial treatment period is scarce and inconclusive. We report our experience in the management of 18 consecutive patients with AHA admitted to two Haemophilia centres in Italy. We managed the first 11 according to current guidelines (e.g., with conventional aPCC doses until bleeding resolution). Then, we decided to prolong the treatment beyond bleeding resolution with lower doses of the same concentrate (short-term prophylaxis) in the 7 additional patients. In these patients, the treatment was continued for as long as the titre of FVIII inhibitor was found to decrease by at least 50% when compared to the baseline one. We observed six relapses of bleeding in patients in whom aPCC was confined to the treatment of the qualifying bleeding episode, and none in patients to whom lower doses were administered until the pre-specified decrease in the titre of FVIII inhibitor was achieved. No patients experienced thrombotic complications during the study period. Prolonging the treatment with lower doses of aPCC beyond the initial phase in patients with AHA in whom the titre of FVIII inhibitor is still high is likely to safely prevent further bleeding complications.
Keywords: Acquired haemophilia; Activated prothrombin complex concentrate; Bleeding; Prophylaxis.
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