and bleeding disorders should be interfere with the function of specific clotting proteins section has been updated in consultation Diagnostic undertaken only by or in consultation including factor VIII, factor IX and less commonly, other with the Australian Haemophilia Centre Criteria with haemophilia treatment centres. coagulation factors. Inhibitors can be allo-antibodies as Directors’ Organisation to reflect the When indicated, intravenous occurs in congenital haemophilia in response to infused findings of more recently published immunoglobulin (IVIg) only forms part product, or auto-antibodies as occurs in acquired evidence on disease presentation, of the management of these complex haemophilia which may be seen in the setting of other progression and management. patients, with additional haemostatic autoimmune disease, malignancy, post-partum or in support required. response to certain drugs. The incidence of acquired IVIg may be considered in the following cases is very low and usually respond to steroids circumstances: and IVIg would only usually be considered in the Inhibitors to factor VIII (FVIII) in setting of ongoing bleeding. Patients may present with haemophilia A and inhibitors to factor abnormal bleeding which can be severe and life IX (FIX) in haemophilia B, especially in threatening, and in these circumstances treatment is best cases where there has been failure of co-ordinated in association with Haemophilia Treatment immune tolerisation and poor Centres. The presence and level of inhibitor should be response to recombinant factor VIIa or confirmed where possible by a factor specific Bethesda factor eight inhibitor bypassing activity assay. Where laboratory confirmation is not able to be (FEIBA) — only as part of the easily performed, as in acquired von Willebrand disease, Malmö protocol for immune tolerance significantly reduced response to infused factor induction. concentrate or demonstration of new onset of reduced Autoimmune acquired von Willebrand clotting factors levels can be used to make the diagnosis. syndrome — correction of FVIII and von Willebrand factor levels for the management of bleeding and before invasive procedures, except cases associated with IgM paraprotein where response is unlikely. Use is indicated in failure to respond to chemotherapy/immunosuppressants or where there is insufficient time for chemotherapy/immunosuppressants
and Diagnostic Criteria bleeding disorders should be undertaken only by or in consultation with haemophilia treatment centres. When indicated, intravenous immunoglobulin (IVIg) only forms part of the management of these complex patients, with additional haemostatic support required. IVIg may be considered in the following circumstances: Inhibitors to factor VIII (FVIII) in haemophilia A and inhibitors to factor IX (FIX) in haemophilia B, especially in cases where there has been failure of immune tolerisation and poor response to recombinant factor VIIa or factor eight inhibitor bypassing activity (FEIBA) — only as part of the protocol for immune tolerance induction. Autoimmune acquired von Willebrand syndrome — correction of FVIII and von Willebrand factor levels for the management of bleeding and before invasive procedures, except cases associated with IgM paraprotein where response is unlikely. Use is indicated in failure to respond to chemotherapy/immunosuppressants or where there is insufficient time for chemotherapy/immunosuppressants interfere with the function of specific clotting proteins including factor VIII, factor IX and less commonly, other coagulation factors. Inhibitors can be allo-antibodies as occurs in congenital haemophilia in response to infused product, or auto-antibodies as occurs in acquired haemophilia which may be seen in the setting of other autoimmune disease, malignancy, post-partum or in response to certain drugs. The incidence of acquired cases is very low and usually respond to steroids and IVIg would only usually be considered in the setting of ongoing bleeding. Patients may present with abnormal bleeding which can be severe and life threatening, and in these circumstances treatment is best co-ordinated in association with Haemophilia Treatment Centres. The presence and level of inhibitor should be confirmed where possible by a factor specific Bethesda assay. Where laboratory confirmation is not able to be easily performed, as in acquired von Willebrand disease, significantly reduced response to infused factor concentrate or demonstration of new onset of reduced clotting factors levels can be used to make the diagnosis. section has been updated in consultation with the Australian Haemophilia Centre Directors’ Organisation to reflect the findings of more recently published evidence on disease presentation, progression and management. Malmö
Acquired-haemophilias-and-congenital-haemophilia-with-inhibitors2017.docx