von Willebrand disease and pregnancy: a practical approach for the diagnosis and treatment.
von Willebrand disease is caused by either a quantitative or qualitative defect in von Willebrand factor (VWF). Patients may have extensive mucosal bleeding (because of platelet dysfunction) and prolonged bleeding after surgery (because of factor VIII deficiency). Up to 6 different subtypes of the disease have been described, and diagnosis is based on clinical suspicion and laboratory confirmation. Accurate diagnosis is of paramount importance because therapy will vary according to the subtype. Bleeding complications during pregnancy are more frequent when levels of the von Willebrand ristocetin cofactor assay and factor VIII levels are <50 IU/dL. In such cases, therapy before any invasive procedure or delivery must be instituted. The mainstays of therapy are desmopressin and plasma concentrates that contain von Willebrand factor. Delayed postpartum hemorrhage may occur, despite adequate prophylaxis. Frequent monitoring and continued prophylaxis and/or treatment are recommended for at least 2 weeks after delivery.
Pacheco
Luis D
LD
Divisions of Maternal Fetal Medicine and Surgical Critical Care, Departments of Obstetrics & Gynecology and Anesthesiology, The University of Texas Medical Branch, Galveston, TX, USA. ldpachec@utmb.edu
Costantine
Maged M
MM
Saade
George R
GR
Mucowski
Sara
S
Hankins
Gary D V
GD
Sciscione
Anthony C
AC
D016913:Blood Component Transfusion; D002881:Chromosomes, Human, Pair 12; D003266:Continuity of Patient Care; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D036861:Delivery, Obstetric; D005169:Factor VIII / Q000627:therapeutic use; D005260:Female; D006490:Hemostatics / Q000627:therapeutic use; D006801:Humans; D009154:Mutation; D006473:Postpartum Hemorrhage / Q000517:prevention & control; D049590:Postpartum Period; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000175:diagnosis* / Q000628:therapy*; D012310:Ristocetin; D014592:Uterine Hemorrhage / Q000517:prevention & control; D014842:von Willebrand Diseases / Q000145:classification / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D014841:von Willebrand Factor / Q000627:therapeutic use
D006490:Hemostatics; D014841:von Willebrand Factor; D012310:Ristocetin; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin