Abstract
Hemophilia B is a rare X-linked disorder that may cause dramatic bleeding. Women account
for only 3.2% of those clinically affected. The X-linked inheritance frequently delays
the diagnosis in women and may expose the patient to an increased risk of adverse
events. There is limited experience with these patients during labor and delivery.
A 28-year-old primiparous woman with hemophilia B (bleeding phenotype) delivered a
male infant by an unplanned cesarean delivery under general anesthesia following treatment
with factor IX and normalization of her coagulation parameters, guided by thromboelastography.
Postpartum vaginal bleeding required transfusion of two units of packed red blood
cells. Factor IX supplementation continued for one week. Once diagnosed with hemophilia
B, a multidisciplinary approach and advanced antenatal planning can increase the likelihood
of a safe delivery. Neuraxial approaches and cesarean delivery are recommended only
after normalization of the coagulation profile. The male fetus of a hemophilia A or
B patient requires special attention. Operative vaginal delivery and invasive fetal
monitoring should be avoided. Thromboelastography is an excellent technique to assess
parturients with bleeding disorders or peripartum hemorrhage and may be underused.
Keywords
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Article info
Publication history
Published online: June 06, 2011
Accepted:
April 5,
2011
Identification
Copyright
© 2011 Elsevier Ltd. Published by Elsevier Inc. All rights reserved.
