Hemophilia A and B vs Von Willebrand Disease
Hemophilia A and B and Von Willebrand Disease are easy to mix up on the boards. Here's a side-by-side comparison — presentation, workup, imaging, and first-line treatment — drawn from our full outlines.
Hemophilia A and B vs Von Willebrand Disease at a glance
- Hemophilia A and B: X-linked recessive bleeding disorders from factor VIII (A) or factor IX (B) deficiency — joint and deep-tissue bleeding.
- Von Willebrand Disease: Most common inherited bleeding disorder — quantitative or qualitative defect in von Willebrand factor (vWF) causing mucocutaneous bleeding.
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Side-by-side comparison
| Feature | Hemophilia A and B | Von Willebrand Disease |
|---|---|---|
| At a glance | X-linked recessive bleeding disorders from factor VIII (A) or factor IX (B) deficiency — joint and deep-tissue bleeding. | Most common inherited bleeding disorder — quantitative or qualitative defect in von Willebrand factor (vWF) causing mucocutaneous bleeding. |
| Classic presentation | Male infant with prolonged bleeding after circumcision; toddler with knee hemarthrosis after minor trauma; X-linked family history.; Hemarthrosis (joint bleeding) — knees, elbows, ankles most common; warm, swollen, painful joint with limited range of motion; Deep muscle hematomas (iliopsoas, calf, forearm) — risk of compartment… | Adolescent female with heavy menses, recurrent epistaxis, easy bruising, and a family history of bleeding.; Mucocutaneous bleeding: epistaxis (often prolonged, recurrent, requiring intervention), gingival bleeding, easy bruising; Heavy menstrual bleeding (menorrhagia) — common presenting feature in women, may begin at menarche;… |
| Workup / key labs | Prolonged PTT correcting with mixing study + reduced factor VIII (hemophilia A) or factor IX (hemophilia B) with normal vWF. Family history and genetic testing supportive.; PTT prolonged, PT normal, platelet count normal, bleeding time/PFA-100 normal; Mixing study — PTT corrects with 1:1 mix of patient and normal plasma (factor… | Personal and/or family bleeding history + reduced vWF antigen and/or activity (typically <30 IU/dL; 30-50 IU/dL suggestive in symptomatic patients). Subtype determined by multimer analysis and specialized assays.; Initial screen: CBC (platelets normal except type 2B), PT normal, PTT may be prolonged (if low factor VIII), platelet… |
| Imaging | MRI for joint and muscle bleeding (more sensitive than X-ray for acute hemarthrosis); CT head urgently for any head trauma or neurologic symptoms; Ultrasound for soft tissue hematoma assessment | Echocardiogram if Heyde syndrome (acquired vWD + GI bleeding from angiodysplasia + severe AS) suspected; Endoscopy for unexplained GI bleeding (angiodysplasia) |
| First-line treatment | Acute bleeding: factor replacement — recombinant factor VIII for hemophilia A (e.g., octocog alfa, efmoroctocog alfa [extended half-life]), recombinant factor IX for hemophilia B (e.g., nonacog alfa, albutrepenonacog alfa, eftrenonacog alfa); Dosing: minor bleed target 30-50% activity, major bleed 80-100%, surgery 80-100%; 1 unit/kg of… | Desmopressin (DDAVP) — releases vWF and factor VIII from endothelial stores; first-line for type 1 and most type 2A; IV, intranasal, or SC; CONTRAINDICATED in type 2B (worsens thrombocytopenia) and ineffective in type 3; vWF/factor VIII concentrate — humate-P, Wilate (plasma-derived), or recombinant vWF (vonicog alfa, Vonvendi); used in… |
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