Acquired haemophilia A management remains a challenge
The best way to manage acquired haemophilia A in older people is unclear, according to a systematic review by specialists in France (Semin Hematol 2018;55:197-201). Their analysis of 80 publications describing 159 patients found a that a range of interventions was used and their effectiveness is difficult to assess. Mortality was high and depended on how quickly the disorder was diagnosed and treated.
A series of 6 cases (four aged >90) recently reported from Japan (and therefore unlikely to be included in the review) notes that acquired haemophilia A can be readily diagnosed provided it is recognised (Case Rep Hematol 2018 Nov 13; doi: 10.1155/2018/6757345). Treatment included rFVIIa, activated prothrombin complex concentrate and fresh frozen plasma. Regimes for immunosuppression included prednisone as monotherapy or in combination with cyclophosphamide, cyclosporine or rituximab. This approach failed in one patient, in whom the anti-FVIII inhibitor level was 240 BU/ml.
The diagnosis and management of acquired haemophilia A is also discussed in a recent review (Drugs 2018;78:1861-1872).