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Von Willebrand Disease

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In one line

·Von Willebrand disease is a failure of the glue that helps platelets stick to injured blood vessels, making bleeding last longer than it should.

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Normal physiology

·Von Willebrand factor (VWF) is a large sticky protein made by endothelial cells (the cells lining blood vessels) and stored in platelets. When a blood vessel is injured, VWF is released and unfolds into long strands that stick to the exposed collagen (structural protein) in the vessel wall. Platelets then attach to these VWF strands, forming the first layer of a blood clot. VWF also carries and protects factor VIII (a clotting protein needed for the next steps of clot formation).

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What goes wrong

·In von Willebrand disease, the body either doesn't make enough von Willebrand factor, or the factor is made incorrectly so it doesn't work well. Sometimes the protein is released too quickly from storage, or it breaks down too fast in the bloodstream. The result is that platelets can't stick properly to injured blood vessel walls, and factor VIII levels drop because it's not protected, so both the initial platelet plug and the later clot formation are weak.

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Hallmark signs

·Easy bruising
·Frequent nosebleeds lasting more than 10 minutes
·Heavy menstrual bleeding requiring pad or tampon change every 1–2 hours
·Prolonged bleeding after dental extraction or surgery
·Blood in stool or black tarry stools
·Joint swelling and pain
·Excessive bleeding after minor cuts lasting more than 15 minutes
·Bleeding in the mouth or gums with routine tooth brushing

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Red flags · escalate now

·Hemarthrosis (joint bleeding) or deep muscle a pooled collection of blood (hematoma) suggesting severe type 3 VWD with very low factor VIII
·Postpartum bleeding (hemorrhage) requiring transfusion or ICU admission
·Signs of hypovolemic shock (tachycardia, low blood pressure (hypotension), altered mental status) from acute blood loss
·New onset bleeding in older adult suggesting acquired von Willebrand syndrome from lymphoma, myeloproliferative disorder, or aortic narrowing (stenosis)

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Workup

·Von Willebrand factor antigen (VWF:Ag)
·Von Willebrand factor activity (VWF:RCo or VWF:GPIbM)
·Factor VIII activity
·VWF multimer analysis
·Complete blood count (CBC)
·Bleeding time or PFA-100 (platelet function analyzer)
·Blood type (ABO typing)
·VWF:FVIII binding assay

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Treatment

·Desmopressin (DDAVP) given intravenously, subcutaneously, or intranasally before procedures
·VWF/factor VIII concentrate infusion for major bleeding or surgery
·Tranexamic acid oral or intravenous
·Hormonal contraceptives (combined oral pills, levonorgestrel IUD, or depot medroxyprogesterone) for heavy menstrual bleeding
·Avoidance of antiplatelet drugs (aspirin, NSAIDs, clopidogrel) and anticoagulants unless absolutely necessary
·Red blood cell transfusion for symptomatic anemia from acute bleeding (hemorrhage)

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NCLEX trap

·Any invasive procedure including dental work can cause significant bleeding in VWD patients. Preventive (Prophylactic) desmopressin or factor replacement must be given before dental procedures, even simple cleanings, based on disease severity and type.
·Platelet count is usually normal in VWD because the problem is not the number of platelets but how well they stick together. Von Willebrand factor helps platelets adhere to damaged blood vessels. VWD diagnosis requires specific von Willebrand factor testing, not just a platelet count.
·Desmopressin works well only for Type 1 VWD and some Type 2 variants. Type 2B VWD can worsen with DDAVP causing a low platelet count (thrombocytopenia) (low platelets), and Type 3 VWD patients have no stored factor to release, so they need factor replacement concentrates instead.
·Even mild VWD patients should apply direct pressure for at least 15 to 20 minutes for cuts, use ice packs, and consider topical thrombin or tranexamic acid mouthwash for oral bleeding. Seemingly minor bleeding can become prolonged and significant without proper first aid.
·Fresh frozen plasma contains very low levels of von Willebrand factor and requires large volumes that risk fluid overload. The correct treatment is von Willebrand factor concentrates (like Humate-P or Wilate) which provide concentrated factor in small volumes and include factor VIII.
·Von Willebrand factor and factor VIII levels naturally rise during pregnancy, often to normal ranges by the third trimester, reducing bleeding risk during delivery. However, levels drop rapidly postpartum, so the highest bleeding risk is actually 24 to 48 hours after delivery, not during pregnancy.

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