What bleeding disorder, such as von Willebrand disease or coagulopathy, is consistent with easy bruising, menorrhagia, and a high risk of maternal hemorrhage during delivery in a pregnant patient?

Von Willebrand Disease

Von Willebrand disease (VWD) is the blood disorder that presents with easy bruising, menorrhagia, and carries high risk for maternal hemorrhage during delivery. 1, 2, 3

Clinical Presentation

VWD is the most common inherited bleeding disorder and manifests with a characteristic mucocutaneous bleeding pattern that includes: 1, 2, 4

• Easy bruising (often >5 cm, occurring 1-2 times per month) 5
• Menorrhagia (heavy menstrual bleeding, often since menarche) 2, 5, 4
• Epistaxis (frequent nosebleeds, 1-2 times per month) 2, 5
• Bleeding after dental procedures or surgery 1, 2
• Maternal history of bleeding disorder (autosomal inheritance pattern affecting males and females equally) 1

Women are disproportionately affected by bleeding challenges in VWD despite equal prevalence, suffering from sex-specific symptoms including menorrhagia, reproductive problems, recurrent miscarriage, and peripartum hemorrhage. 4

Pregnancy-Related Hemorrhage Risk

Pregnant women with VWD face significantly elevated risks for maternal hemorrhage: 3, 4

• Primary and secondary postpartum hemorrhage (PPH) 6, 3, 4
• Placental abruption 6
• Vaginal bleeding during pregnancy 6
• Anemia requiring transfusions 4
• Bleeding complications during delivery 3, 4

The prevalence of VWD among women with menorrhagia is approximately 11-16%, making it a critical diagnosis to consider in women presenting with heavy menstrual bleeding. 5, 7

Pathophysiology

VWD causes bleeding through dual mechanisms: 1

• Impaired platelet adhesion at sites of vascular injury due to deficient or dysfunctional von Willebrand factor (VWF) 1, 2
• Secondary factor VIII deficiency because VWF carries and stabilizes factor VIII in circulation 1, 2

Laboratory Findings

Characteristic laboratory abnormalities include: 1

• Prolonged aPTT with normal PT (indicating intrinsic pathway defect) 1
• Mildly decreased factor VIII activity (secondary to VWF deficiency) 1
• Normal platelet count (distinguishing it from thrombocytopenia) 1
• Decreased VWF antigen and ristocetin cofactor activity 5

Management During Pregnancy and Delivery

For pregnant women with VWD, management requires maintaining VWF activity levels ≥50 IU/dL during delivery and the postoperative period: 8

Hemostatic Agents

• Desmopressin 0.3 μg/kg IV (maximum 28 μg) is first-line therapy for Type 1 VWD, raising endogenous VWF and factor VIII levels 3-6 fold within 30-90 minutes 1, 8, 9
• VWF/factor VIII concentrates for patients who don't respond to desmopressin or have severe disease 8, 9, 3
• Tranexamic acid as adjunctive therapy to reduce bleeding risk 6, 5

Delivery Planning

All moderately or severely affected women should: 3

• Obtain coagulation factor levels at minimum in the third trimester 3
• Plan delivery at a center with hemostasis expertise 3
• Anticipate the need for hemostatic agents peripartum 3
• Monitor VWF activity levels throughout pregnancy (levels naturally rise during pregnancy but may remain insufficient) 4

Critical Pitfalls to Avoid

Women with VWD and menorrhagia often face delayed diagnosis because bleeding symptoms are misunderstood as gynecologic rather than hematologic problems. 4, 7 This leads to:

• Unnecessary surgical interventions (D&C, endometrial ablation, hysterectomy) performed without addressing the underlying bleeding disorder 5
• Increased risk of hemorrhagic complications during these procedures 5
• Continued bleeding and diminished quality of life 5, 4

The key is recognizing the constellation of lifelong mucocutaneous bleeding, maternal family history, and post-procedural bleeding as characteristic of VWD rather than isolated gynecologic pathology. 1