Adolescent with heavy menstrual bleeding, borderline von Willebrand studies, and upcoming tonsillectomy
A 14-year-old girl presents with heavy menstrual bleeding since menarche 10 months ago. Periods last 8–10 days with clots, and she soaks a pad hourly for the first 2 days. She has fatigue, easy bruising, and two prolonged nosebleeds but no other bleeding. She takes ibuprofen for cramps. Hemoglobin is 8.4 g/dL, ferritin 4 ng/mL, platelets 250 × 10^9/L, PT/INR and aPTT normal. PFA-100 closure times are prolonged. VWF antigen is 48 IU/dL, VWF ristocetin cofactor 42 IU/dL, and factor VIII 52 IU/dL; repeat testing 3 months later is similar. She is blood group O. She is scheduled for tonsillectomy in 6 weeks for recurrent tonsillitis. Her mother asks whether she can just take a pill to stop her periods.
1. How do you classify her bleeding disorder, and which confounders affect interpretation of the von Willebrand results?
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The picture is most consistent with type 1 von Willebrand disease or the related category of low von Willebrand factor, rather than a platelet function defect. She has mucocutaneous bleeding, prolonged PFA-100 closure times, and VWF antigen and ristocetin cofactor in the 40s with a normal factor VIII. The VWF ristocetin cofactor-to-antigen ratio is above 0.7, which argues against type 2. Type 3 is excluded by detectable antigen.
Several confounders matter. Blood group O lowers VWF by roughly 25%, so a value of 48 IU/dL in a group O adolescent may still be physiologically low but is not diagnostic by itself. Values between 30 and 50 IU/dL with bleeding are often managed as low VWF; a formal diagnosis usually requires a consistent level below 30 IU/dL or a compatible pattern with a convincing bleeding score. Acute bleeding, inflammation, exercise, and pregnancy raise VWF, so testing should be repeated when stable, as it was here. Iron deficiency itself does not cause prolonged PFA-100 times, but anemia can worsen fatigue and bleeding tolerance.
The reproducible low values plus a significant bleeding history justify treating her as having a mild VWD phenotype, with hematology follow-up and a repeat panel before surgery.
2. What is your immediate management of the heavy menstrual bleeding and iron deficiency, and what should she stop?
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She needs control of bleeding and correction of iron deficiency. Stop ibuprofen: NSAIDs impair platelet function and can worsen gastrointestinal and menstrual bleeding. Use acetaminophen or a hormonal approach for cramps. For acute heavy bleeding, tranexamic acid at about 15–25 mg/kg per dose every 8 hours during menses, with a usual maximum of 1–1.5 g per dose, is first-line and can reduce flow substantially. A combined oral contraceptive taken continuously or cyclically, or oral norethindrone, can stabilize the endometrium; the choice depends on her preferences, thrombosis risk, and ability to adhere.
Start oral iron at 3–6 mg/kg/day of elemental iron; if she cannot tolerate it or the hemoglobin is falling, intravenous iron is reasonable. Transfusion is reserved for symptomatic severe anemia or hemodynamic compromise. Check a pregnancy test and thyroid function if not already done, and screen for other causes of heavy bleeding. Reassess hemoglobin in 2–4 weeks. Her mother's question about 'a pill to stop periods' should be answered directly: hormonal therapy can lighten or suppress periods, but it does not replace hemostatic planning for surgery or iron repletion.
3. Plan the perioperative hemostatic management for tonsillectomy. What would make you choose desmopressin versus a VWF-containing concentrate?
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Tonsillectomy is a high-risk mucosal operation, and bleeding can be delayed for 7–10 days. The plan should be coordinated among hematology, otolaryngology, and anesthesia. First, obtain a preoperative VWF panel and consider a desmopressin trial: give 0.3 mcg/kg intravenously and measure VWF antigen, VWF activity, and factor VIII at baseline and 1 hour. A brisk rise to hemostatic levels predicts a response. If she responds, use desmopressin 0.3 mcg/kg intravenously 30–60 minutes before surgery, with careful fluid restriction and sodium monitoring; repeated doses cause tachyphylaxis and hyponatremia. Add tranexamic acid at 15–25 mg/kg every 8 hours, orally or intravenously, and continue for 7–10 days after surgery.
Choose a VWF-containing factor VIII concentrate instead if she does not respond to desmopressin, if the baseline VWF activity is very low, or if the surgical team anticipates a difficult dissection. Avoid aspirin and NSAIDs after surgery. Provide a written bleeding action plan, and instruct the family to call for bleeding, black stools, or inability to drink. Do not rely on a normal aPTT to exclude a bleeding risk.