Severe
factor XI deficiency is an injury-related
bleeding disorder. The risk of excessive post-partum
hemorrhage in affected women has so far been evaluated in a relatively small number of patients and it is uncertain whether prophylactic treatment with fresh frozen plasma or
factor XI concentrate is needed during or after vaginal or cesarean delivery. We retrospectively analyzed
bleeding manifestations related to vaginal and/or cesarean deliveries in a cohort of 62 women with
factor XI activity < 17 U/dl and evaluated whether replacement
therapy is essential. Fifty-one women had 139 vaginal deliveries, six women had 13 cesarean deliveries, and five women had seven vaginal as well as five cesarean deliveries. Forty-three of the 62 women (69.4%) never experienced post-partum
hemorrhage during 93 deliveries (85 vaginal, eight cesarean).
Hemorrhage occurred in 19 women, which in six women accompanied each one of their 17 vaginal deliveries. Post-partum
hemorrhage had no relationship with the abnormal genotype that caused
factor XI deficiency nor with
factor XI level. These observations suggest that the use of fresh frozen plasma or
factor XI concentrate during and/or after vaginal delivery is not mandatory in women with severe
factor XI deficiency and can be reserved for patients who develop excessive
hemorrhage. For women requiring
cesarean section it appears that the same policy can be advocated but more observations are needed.