Table 2

Recommendations for MHA

1. Patients with suspected MHA should be referred to a specialized hemophilia treatment center; 
2. Perform genetic testing to identify patients with mutations potentially at risk for inhibitor; 
3. Perform a desmopressin challenge in all patients unless a contraindication exists; 
4. Use desmopressin where possible, and use caution with high-dose/prolonged courses of FVIII replacement therapy, especially in patients with mutations associated with inhibitor development; 
5. Test for inhibitor after 4-6 wk from intensive treatment with FVIII concentrates, before surgery, or at least every 6 or 12 mo if sporadically treated with FVIII concentrates; 
6. Record accurately the progressive number of ED to anticipate the onset of inhibitor, especially in patients with high-risk mutations. 
1. Patients with suspected MHA should be referred to a specialized hemophilia treatment center; 
2. Perform genetic testing to identify patients with mutations potentially at risk for inhibitor; 
3. Perform a desmopressin challenge in all patients unless a contraindication exists; 
4. Use desmopressin where possible, and use caution with high-dose/prolonged courses of FVIII replacement therapy, especially in patients with mutations associated with inhibitor development; 
5. Test for inhibitor after 4-6 wk from intensive treatment with FVIII concentrates, before surgery, or at least every 6 or 12 mo if sporadically treated with FVIII concentrates; 
6. Record accurately the progressive number of ED to anticipate the onset of inhibitor, especially in patients with high-risk mutations. 
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