• Peter A Kouides.
• Mary M. Gooley Hemophilia Center, Inc. University of Rochester School of Medicine, Rochester, New York, USA.
• Curr. Opin. Hematol. 2015 Sep 1; 22 (5): 397-405.

Purpose Of ReviewThe morbidity and mortality of postpartum hemorrhage (PPH) in women with an underlying bleeding disorder requires vigilance by the hematologist.Recent FindingsRecent 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-FviiiC or VWF deficient, adjunctive antifibrinolytic therapy, e.g., tranexamic acid, appears to be in order as a prophylactic measure.SummaryWomen 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.

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