An update on the management of bleeding disorders during pregnancy

The morbidity and mortality of postpartum hemorrhage (PPH) in women with an underlying bleeding disorder requires vigilance by the hematologist.

Recent studies suggest that women with underlying bleeding disorders may be 'undertreated' at the time of delivery in aiming for too low a target elevation that historically per numerous society guidelines has aimed for VWF/FVIII:C levels to be 'only' greater than 50% when in actuality the levels should be akin to what is achieved in a normal pregnancy. The result appears to be an increase in the rate and degree of PPH. In this context, although recent studies imply DDAVP is well tolerated, DDAVP may not be appropriate because it may not raise the levels into the normal supraphysiological range nor maintain it for several days. Particularly in women with rare bleeding disorders, i.e., non-

C or VWF deficient, adjunctive antifibrinolytic therapy, e.g., tranexamic acid, appears to be in order as a prophylactic measure.

Women with an underlying bleeding disorder appear to be at a heightened risk for PPH if the respective coagulation factor level is not appropriately replaced to the level that is physiologically achieved in a normal pregnancy. Furthermore, there appears to be underuse of tranexamic acid for prophylaxis of PPH in this population.