Evaluation and Management of a 14-Year-Old with Heavy Menstrual Bleeding, Easy Bruising, and Prolonged Bleeding Despite Normal Labs
This adolescent requires immediate specialized testing for von Willebrand disease (VWD) and platelet function disorders, as standard coagulation tests (PT/aPTT) do not detect these conditions, which are the most common bleeding disorders in adolescent females with heavy menstrual bleeding. 1, 2, 3
Critical Understanding: "Normal Labs" Are Insufficient
- Standard PT and aPTT testing does not detect VWD, Factor XIII deficiency, or platelet function disorders, which are the most likely diagnoses in this clinical scenario 1, 2, 4
- Up to 20% of adolescents with heavy menstrual bleeding have an underlying bleeding disorder, with VWD being the most common inherited bleeding disorder (prevalence approximately 1 in 1000) 3, 5, 6
- Normal platelet count does not rule out qualitative platelet defects that can cause significant bleeding manifestations 2
Immediate Diagnostic Workup Required
Essential Specialized Testing (Beyond Standard Coagulation Studies)
VWD-specific testing must include: 1, 2, 3
- VWF antigen (VWF:Ag)
- VWF ristocetin cofactor activity (VWF:RCo)
- Factor VIII coagulant activity (FVIII:C)
- Critical timing: Testing should be performed during menstruation to capture the lowest VWF levels 7
Additional required tests: 1, 2, 3
- Complete blood count with peripheral blood smear review (to assess platelet morphology and anemia from blood loss)
- Serum ferritin (to evaluate iron deficiency from chronic blood loss)
- Fibrinogen level (more sensitive than PT/aPTT for detecting coagulopathy)
- Platelet function studies if VWD testing is normal but clinical suspicion remains high
Factor XIII Deficiency Consideration
- Factor XIII deficiency causes significant bruising and bleeding but is not detected by standard PT/aPTT screening 1, 2
- Requires specific Factor XIII assay if other testing is unrevealing
Clinical Assessment Details to Document
Quantify menstrual blood loss: 3, 6
- Use Pictorial Bleeding Assessment Chart (PBAC) score—a score >100 increases diagnostic sensitivity to 95%
- Document duration of bleeding (>7 days is abnormal)
Associated bleeding symptoms to specifically query: 1, 3, 4
- Epistaxis (frequency, duration, need for medical intervention)
- Gingival bleeding with tooth brushing
- Bleeding after dental procedures or minor trauma
- Family history of bleeding disorders, particularly in female relatives with heavy menstrual bleeding
Physical examination priorities: 5, 4
- Hemodynamic stability assessment (orthostatic blood pressure and pulse)
- Mucocutaneous bleeding patterns (suggests platelet dysfunction or VWD)
- Bruising location and pattern (hemarthroses or deep hematomas suggest coagulopathy)
Immediate Management Strategy
Acute Bleeding Control
First-line medical management (surgery reserved for non-responders): 3, 5
- Tranexamic acid (antifibrinolytic): Reduces menstrual blood loss by up to 50%, available in oral and IV formulations
- NSAIDs (mefenamic acid or naproxen): First-line for 5-7 days during menstruation only
- High-dose estrogen therapy may be necessary for severe acute bleeding with transfusion support
Maintenance Therapy After Acute Control
- 52-mg levonorgestrel intrauterine device has superior efficacy for heavy menstrual bleeding and is safe in adolescents with bleeding disorders
- Combined oral contraceptive pills for cycle control
- Oral or injectable progestins
Non-hormonal maintenance: 3, 5
- Continued tranexamic acid during menses if hormonal therapy contraindicated
Critical Pitfalls to Avoid
- Do not assume normal PT/aPTT rules out bleeding disorders—this is the most common diagnostic error in this population 1, 2, 4
- Do not delay hematology referral while waiting for specialized test results if bleeding is severe 1, 3
- Do not perform VWD testing outside of menstruation as levels may be falsely elevated 7
- Do not overlook iron deficiency anemia which requires concurrent treatment 3, 5
Mandatory Hematology Referral Indications
Immediate consultation required for: 1, 2, 3
- Interpretation of specialized VWD testing
- Platelet function studies (light transmission aggregometry, flow cytometry)
- VWD multimer analysis if initial testing suggests VWD
- Coordination of desmopressin (DDAVP) trial or factor replacement therapy if VWD confirmed
- Preoperative planning for any future surgical procedures
Expected Outcomes and Follow-Up
- If VWD is diagnosed: Treatment with desmopressin or VWF concentrates combined with hormonal therapy typically provides excellent bleeding control 5, 7
- If platelet dysfunction identified: Platelet dysfunction typically improves within 2-4 weeks of treating underlying conditions, though inherited disorders require ongoing management 2
- Anemia, transfusion needs, and hospitalization can be avoided with prompt recognition and treatment 6