A low initial inhibitor titre and a short interval between the ap

A low initial inhibitor titre and a short interval between the appearance of the inhibitor and the start of therapy seem to be positive predictive factors. The problems of infectious complications and therapy-related mortality were addressed, but

data are scanty. In a randomized prospective multicentre trial [26], 31 patients with newly diagnosed acquired haemophilia were treated with prednisone 1 mg kg day−1 for 3 weeks; 20 non-responders were randomized: four patients Trametinib cost with prednisone (1 mg kg day−1); six patients with cyclophosphamide 2 mg kg day−1; 10 patients with prednisone + cyclophosphamide for additional 6 weeks. The inhibitor disappeared in three patients (75%) treated with prednisone and in eight patients (50%) treated with cyclophosphamide or cyclophosphamide + prednisone. No information on the follow-up was given. In the Italian study [3], 65 of 90 patients were evaluable for the immunosuppressive therapy. Three patients died

before starting treatment, one because of bleeding and two for reasons of the underlying disease. Eight patients with a low inhibitor titre (<10 BU) did not receive immunosuppressive therapy; three of them died because of bleeding complications. Information relevant to the response to the immunosuppressive therapy was missing in 14 patients. Results of the initial immunosuppressive therapy: complete remission 46 (70.7%), partial remission 13 (20%), failure 6 (9.3%). Four patients in partial remission Pregnenolone achieved a complete remission after discontinuation of treatment. selleck products The other patients including the failures received alternative treatments (Table 4). Patients with low (<10 BU) or high (>10 BU) inhibitor titre did not differ in the rate of complete remission (30 and 22 patients respectively). Eleven patients (21.1%) relapsed; eight were rescued with additional therapy, one patient died because of bleeding and two achieved

a spontaneous complete remission. Rituximab, an anti-CD 20 monoclonal antibody, has been used as salvage therapy. Sperr et al. compared Rituximab and prednisone + cyclophosphamide in 42 and 44 patients respectively reported in various studies in the literatures [27]. Results were similar: complete remission (CR) rate 78.6% and 84.1% without difference between patients who had (75%) or had not received previous treatment with other immunosuppressive drugs. The median treatment duration to CR was 8.3 and 6.3 weeks and the probability of CR at 2 years 66% and 94% with a plateau in the Kaplan–Mayer curve. The authors concluded that the use of Rituximab should be limited to failure of first/second line therapy. Few patients were treated with cyclosporine A or 2-chlorodeoxyadenosine. Immune tolerance is an accepted and effective treatment of haemophilic patients with inhibitor, but has been rarely applied in acquired haemophilia.

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