Pregnancy in subtype 2B VWD

CASAIS P; MESCHENGIESER SS; KEMPFER AC; WOODS AI; SÁNCHEZ-LUCEROS A; LAZZARI MA

Vancouver

Congreso; XXVII International Congress of the World Federation of Haemophilia; 2006

World Federation of Haemophilia

There are limited reports about pregnancy; delivery and puerperium management in type 2B VWD which regularly result in thrombocytopenia. Vaginal delivery is generally safe, but the incidence of postpartum haemorrhage is 30 %. These bleedings may be severe. The risk of postpartum bleeding complications cannot be predicted with certainty, either by past history of bleeding episodes or by haematological laboratory tests of VWF activity levels. We report a pregnancy in a 28-year-old woman diagnosed as type 2B VWD. She presented scanty mucocutaneous bleeding without familial antecedents, a VWF:Ag 25%, VWF:Rco 12%, FVIII 50%, increased RIPA, lack of high molecular weight multimers and thrombocytopenia. Her response to DDAVP was adequate but with fall in platelet count (25%). A tooth extraction was covered with DDAVP without bleeding. As we expected the factor levels increased (VWF:Ag 160%, VWF:RCo 51%, FVIII 90%) at the control performed at 8month but she presented a platelet count of 51 000/mL. We suggested cesarean section with general anaesthesia because we considered it was the safest option for mother and child. We indicated replacement therapy with Haemate P 40 IU/kg twice daily starting before cesarean section and continuing for 24 h. In addition, platelet concentrates were indicated immediately before surgery but were actually transfused during the surgery and after. She underwent a vertical incision with no bleeding complications during or after surgery. Drainage showed no blood loss in 24 h and was removed. She gave birth to a healthy boy without bleeding symptoms or thrombocytopenia. Citation: Haemophilia 2006; 12 (Suppl 2): 30PO 906