Table III.

Summary of the Italian Association of Haemophilia Centres’ recommendations on inhibitor eradication therapy in acquired haemophilia A⁷

Immunosuppressive therapy must be started as soon as possible, ideally immediately after the diagnosis of acquired haemophilia A

First-line treatment is prednisone (1–2 mg/kg daily) alone or in combination with cyclophosphamide* (1–2 mg/kg daily)

Rituximab (375 mg/m2 once weekly for a total of 4 doses) as monotherapy or in combination with other immunosuppressive drugs is the main agent for second-line therapy in patients who fail to respond to first-line therapy within 8–12 weeks

Rituximab may be indicated as a first-line agent in patients in whom there is a contraindication to immunosuppressive drugs

The combination of immunosuppressive drugs (including cyclosporine) and immunotolerance regimens are possible second-line alternatives in patients failing to respond to first-line immunosuppressive therapy. High doses of immunoglobulins are not indicated as an inhibitor eradication approach

Complete response to eradication therapy requires persistent negative inhibitor titres (<0.6 BU/mL) and normal factor VIII levels (>70%)

Patients with thromboembolic risk factors should receive mechanical and/or pharmacological thromboprophylaxis, particularly in the case of elevated factor VIII levels during or at the end of eradication treatment

^*Cyclophosphamide and other alkylating agents should be avoided in women of childbearing age with acquired haemophilia A.