Acquired haemophilia A Acquired haemophilia B Acquired von Willebrand syndrome Acquired bleeding disorder, other coagulation factors (Prothrombin, factor V, factor VII, factor X, factor XI, and factor XIII) Level of Evidence of probable benefit – more Evidence of probable benefit – more research needed Unchanged and unlikely to change due to Evidence research needed (Category 2a) (Category 2a) the rarity of the condition. Justification of evidence section has been Justification IVIg has a role as part of a second-line immune tolerance drafted in line with the literature. for Evidence protocol involving immunoadsorption and Category immunoglobulin replacement, in addition to immunosuppression and factor replacement, the Malmo protocol (Nilsson et al, 1988 and Berntorp et al, 2000 and Franchini & Lippi, 2008 and Zeitler et al, 1991). There is evidence for the beneficial effect of this protocol in patients with: • Congenital haemophilia A with acquired factor VIII inhibitors • Congenital haemophilia B with acquired factor IX inhibitors • Acquired haemophilia A. IVIg has an accepted role in the reduction of bleeding in patients with acquired von Willebrand syndrome and IgG paraproteins (Collins et al, 2013). IVIg has an uncertain role in the reduction of bleeding in patients with other acquired bleeding disorders
Acquired haemophilia A Acquired haemophilia B Acquired von Willebrand syndrome Acquired bleeding disorder, other coagulation factors (Prothrombin, factor V, factor VII, factor X, factor XI, and factor XIII) Level of Evidence Evidence of probable benefit – more research needed (Category 2a) Evidence of probable benefit – more research needed (Category 2a) Unchanged and unlikely to change due to the rarity of the condition. Justification for Evidence Category IVIg has a role as part of a second-line immune tolerance protocol involving immunoadsorption and immunoglobulin replacement, in addition to immunosuppression and factor replacement, the Malmo protocol (Nilsson et al, 1988 and Berntorp et al, 2000 and Franchini & Lippi, 2008 and Zeitler et al, 1991). There is evidence for the beneficial effect of this protocol in patients with: • Congenital haemophilia A with acquired factor VIII inhibitors • Congenital haemophilia B with acquired factor IX inhibitors • Acquired haemophilia A. IVIg has an accepted role in the reduction of bleeding in patients with acquired von Willebrand syndrome and IgG paraproteins (Collins et al, 2013). IVIg has an uncertain role in the reduction of bleeding in patients with other acquired bleeding disorders Justification of evidence section has been drafted in line with the literature.
Acquired-haemophilias-and-congenital-haemophilia-with-inhibitors2017.docx