What is the management and what questions to ask for a patient with von Willebrand disease (VWD) presenting with melena and lightheadedness?

Management of VWD Patient with Melena and Lightheadedness

This patient requires immediate hemodynamic stabilization, urgent hematology consultation, and VWF/FVIII replacement therapy while simultaneously investigating the source of gastrointestinal bleeding. 1

Initial Assessment Questions

Hemodynamic Status and Bleeding Severity

• Quantify orthostatic symptoms: Can the patient stand without syncope? Document vital signs including orthostatic blood pressure changes to assess volume depletion 1
• Duration and volume of melena: When did black stools start? How many episodes? Any bright red blood per rectum or hematemesis? 1
• Current bleeding elsewhere: Any epistaxis, gingival bleeding, hematuria, or other mucosal bleeding sites? 1

VWD-Specific History

• VWD subtype and baseline factor levels: What type of VWD (1, 2A, 2B, 2M, 2N, or 3)? What are baseline VWF activity and Factor VIII levels? This determines treatment approach 2, 3
• Prior desmopressin response: Has the patient been tested with desmopressin? What was the response in VWF and FVIII levels? Type 2B and Type 3 will not respond 2, 3
• Recent VWF concentrate use: Any prophylactic treatment? When was last dose? 4

Gastrointestinal Risk Factors

• Medication history: NSAIDs, aspirin, anticoagulants, or antiplatelet agents? These must be identified and discontinued 5, 6
• Prior GI bleeding: Any history of peptic ulcer disease, angiodysplasia, or other GI pathology? VWD patients have increased risk of recurrent GI bleeding 7, 8
• Recent procedures or trauma: Any endoscopy, biopsy, or abdominal trauma? 1

Comorbidities Affecting Hemostasis

• Liver disease: Jaundice, ascites, or known cirrhosis? This affects factor synthesis 1
• Thyroid function: Hypothyroidism can worsen VWD bleeding 1
• Recent infections or inflammatory conditions: Can affect VWF levels 8

Immediate Management Algorithm

Step 1: Hemodynamic Resuscitation

• Establish large-bore IV access (two lines minimum) and initiate crystalloid resuscitation 1
• Type and crossmatch blood products: Order packed red blood cells, fresh frozen plasma if needed 4
• Monitor serum sodium before any desmopressin: Hyponatremia is a critical risk, especially with fluid resuscitation 2
• Obtain baseline labs: CBC, comprehensive metabolic panel (including sodium), PT/PTT, Factor VIII level, VWF antigen, VWF activity (ristocetin cofactor), and blood type 1

Step 2: Hemostatic Therapy Selection

For Type 1 VWD with Factor VIII >5% and known desmopressin responders:

• Administer desmopressin 0.3 mcg/kg IV (maximum 20 mcg) over 15-30 minutes 2
• Strict fluid restriction: Limit to 1 liter in 24 hours to prevent hyponatremia 2
• Monitor serum sodium at baseline, 2-4 hours post-dose, and daily 2
• Repeat dosing: Can give every 12-24 hours if needed, but tachyphylaxis occurs after 48 hours 2, 3

For Type 2A, 2B, 2M, 2N, Type 3, or unknown desmopressin response:

• Administer VWF/FVIII concentrate immediately: Do not wait for desmopressin trial in acute severe bleeding 3, 4
• Dosing: 40-60 IU/kg VWF:RCo initially, targeting VWF activity >50 IU/dL and Factor VIII >50 IU/dL 4, 7
• Repeat dosing: Every 12-24 hours based on factor levels and clinical response 4
• Monitor Factor VIII and VWF levels 30 minutes post-infusion, then every 12-24 hours 2, 4

Common pitfall: Type 2B VWD can worsen with desmopressin due to transient thrombocytopenia—always use VWF concentrate in Type 2B 3, 7

Step 3: Adjunctive Hemostatic Measures

• Tranexamic acid 1 gram IV every 6-8 hours (or 25 mg/kg if <70 kg): Particularly effective for mucosal bleeding including GI sources 1, 7
• Proton pump inhibitor IV: High-dose esomeprazole 80 mg bolus then 8 mg/hour infusion for upper GI bleeding 8
• Avoid platelet transfusions unless severe thrombocytopenia present, as they are generally ineffective in VWD 4

Step 4: Source Control and Gastroenterology Consultation

• Urgent GI consultation for endoscopy once hemodynamically stable and Factor VIII >50 IU/dL 1, 7
• Pre-procedure hemostatic coverage: Ensure VWF activity and Factor VIII >50 IU/dL before endoscopy 2, 4
• Post-procedure monitoring: Continue VWF replacement for 3-7 days depending on intervention performed 4, 7

Step 5: Hematology Consultation

• Immediate consultation for all severe bleeding in VWD patients 7
• Discuss: Need for recombinant VWF (if available), duration of replacement therapy, and transition to prophylaxis if recurrent GI bleeding 7, 8

Critical Safety Considerations

Hyponatremia monitoring is mandatory with desmopressin: Check sodium at baseline, 2-4 hours post-dose, 24 hours, and before each subsequent dose. Discontinue if sodium <130 mEq/L 2

Never assume VWD subtype without recent laboratory confirmation: Acquired von Willebrand syndrome can develop, particularly with underlying conditions 5, 6

Avoid aspirin and NSAIDs permanently in VWD patients with GI bleeding history 5, 6

For recurrent GI bleeding in VWD: Consider long-term prophylaxis with VWF concentrate, hormonal therapy (if angiodysplasia-related), or treatment of underlying GI pathology 7, 8