Pregnancy in women with different types of von Willebrand disease

The course and outcome of pregnancy in women with different types of von Willebrand disease (3 type I, 1 subtype IIA, and 1 subtype IIB) are described. In all patients, factor VIII increased and reached normal levels before delivery, whereas the bleeding time remained prolonged; in subtypes IIA and IIB the abnormal multimeric structure of von Willebrand factor remained unchanged. Deliveries were uneventful in all patients, with two spontaneous vaginal deliveries and three cesarean sections, despite the fact that no replacement therapy was given. Hence, the most important determinant of abnormal hemorrhage during delivery is low factor VIII; the prolonged time can be compensated for by meticulous surgical hemostasis and efficient contraction of the uterus. Replacement therapy with plasma derivatives can usually be avoided providing that normal factor VIII levels have been attained at delivery.