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Type 2N von Willebrand Disease in Pregnancy and Postpartum: A Case Report Lauren N Durand, Robert Bona. Yale University Dept of Medicine and Pediatrics, New Haven, CT, USA

Abstract

Background: von Willebrand Disease is the most common congenital bleeding disorder among the general population. Type 2N von Willebrand Disease is an uncommon recessive disorder resulting from genetic mutations at the Factor VIII binding site of von Willebrand Factor, thus FVIII plasma levels are significantly lower than vWF. Given this rapid clearance of unbound FVIII, patients with Type 2N vWD are often compared to patients with mild/moderate hemophilia A both in clinical and laboratory manifestations. In this case, we follow a woman with 2N vWD through her pregnancy journeys and report on treatment to prevent excess bleeding during pregnancy and delivery.   Case: A 26-year-old female was referred to hematology clinic in the setting of new monochorionic, diamniotic twin pregnancy following recent first trimester spontaneous abortion. Genetic testing confirmed the patient as homozygous for type 2N von Willebrand disease (homozygous c.2372C>T:p.Thr791Met). Baseline coagulation protein levels; FVIII 7.1 IU/dl, vWF:Ag 56 IU/dl, vWF:activity 48 IU/dl.  At 17 weeks gestation, the patient presented to the Emergency Room with excessive vaginal bleeding and was found to have a subchorionic hemorrhage in the setting of complex anomalies of one twin (possible limb-body-stalk anomaly). She was treated with vWF/FVIII replacement (Humate-P^Ò) along with local hemostatic measures. Ultimately, after 3 serious re-bleeding episodes, she opted for dilation and evacuation to preserve maternal morbidity/mortality. She was treated post-operatively with vWF/FVIII replacement for 5 days, with minimal bleeding reported. About 3 months later, the patient reported a new pregnancy. Due to the high-risk nature of her pregnancy and poor outcome from the previous 2 pregnancies, treatment was initiated with recombinant vWF replacement (VonvendiÒ) biweekly to maintain hemostasis antepartum. Frequent infusions and inadequate FVIII recovery led to treatment transition to efanesoctocog alfa (AltuviiioÒ) at 17 weeks gestation with goal FVIII trough of >10%. vWF levels were consistently in the normal to low normal range. No bleeding was reported during the pregnancy, and she tolerated the infusions well.  She was transitioned to a vWF/FVIII replacement product (Wilate^Ò) to raise vWF levels at week 38 and a hemostasis delivery plan was created with goal trough levels of >100% FVIII, vWF Activity/Antigen. Planned C-section at 39 weeks delivered a healthy baby with an estimated blood loss of 700 cc. Therapy with Humate-P^Ò  dosed by FVIII units and tranexamic acid continued postpartum for one week. Re-bleeding occurred after discontinuation of treatment and required additional therapy about every 2-3 weeks with Wilate^Ò and tranexamic acid.   Discussion: We report here the successful treatment of an individual with 2N vWD antepartum and postpartum. We chose to use efanesoctocog alfa (AltuviiioÒ) ante partum since FVIII was in a range concerning for bleeding while vWF levels were maintained during pregnancy. A vWF/FVIII concentrate was selected at delivery to raise both FVIII and vWF levels into a range for adequate hemostasis. Specific guidelines for replacement therapy, trough levels and duration of therapy postpartum do not exist. Further investigation and research should be conducted on the bleeding tendencies of patients with 2N von Willebrand Disease, especially surrounding pregnancy and postpartum care.

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