Rare bleeding disorders

There are insufficient data to recommend routine perioperative supplementation of deficient factors in patients with rare bleeding disorders (RBDs).

We suggest that rFVIIa be used in perioperative bleeding due to inherited factor VII deficiency. 2C

If rFVIIa is given to control perioperative bleeding in inherited factor VII deficiency, we suggest lower doses (e.g. 20 to 25 mgkg-1 every 4 to 6 h) than in haemophilia patients with inhibitors. 2C

There are insufficient data to recommend rFVIIa in perioperative bleeding for patients with other RBDs.

There are insufficient data to recommend periprocedural DDAVP or antifibrinolytic drugs in patients with mild RBDs.