Clinician's Corner

A 14-year-old girl was referred for assessment of heavy menses. Menses commenced at the age of 12 years and were unremarkable until the past six months when she noticed an increased amount and duration of bleeding. The menses were regular but had increased from four to seven days of bleeding and she was now using up to 10 pads/day for the first three days. She denied any sexual activity. Past medical history was unremarkable other than for asthma, and a family history could not be obtained because the patient was adopted. Review of systems was positive for fatigue but negative for change in weight, cold intolerance, shortness of breath, easy bruising or prolonged bleeding. On physical examination the patient was pale but in no distress. Vital signs were normal. General physical and external genital examination was normal, with no evidence of bruising. Laboratory investigation showed a hemoglobin concentration of 74 g/L (normal 120 to 153 g/L), mean corpuscular volume and mean corpuscular hemoglobin were both slightly below normal. The platelet count was 392×109/L (normal 150 to 400×109/L). The international normalized ratio (INR) was 0.91 (normal 0.90 to 1.10), and the activated partial thromboplastin time (PTT) was 26 (normal 25.0 to 35.0). Thyroid function tests were normal. The patient's symptoms were well controlled with oral contraceptives but further testing revealed the cause of the menorrhagia. Bleeding time was slightly prolonged at 10 min (normal 2 to 9 min). Factor VIII was normal, von Willebrand factor (vWf) antigen was 0.28 IU/mL (normal 0.50 to 1.50 IU/mL). The ristocetin cofactor (a measure of vWf activity) was 0.36 IU/mL (0.50 to 1.50 IU/mL). Based on these results a diagnosis of von Willebrand's disease (vWD) type I (vWf is quantitatively reduced but not absent) was made. Type 1 vWD accounts for about 85% of all cases of vWD and typically presents with epistaxis, bruising and menorrhagia. The patient was responsive to desmopressin acetate (DDAVP, Ferring, Toronto) with an increase in vWf to 0.85 IU/mL, and ristocetin cofactor to 0.9 IU/mL. Abnormal vaginal bleeding is a common problem encountered by physicians providing health care to adolescent girls. The dilemma is to distinguish those who need investigation for bleeding disorders from those who do not. A careful history with attention to the number of pads used per day and the degree to which they are soiled will determine the degree of bleeding. Six soaked pads per day for three or more days would be considered to be heavy. Dysfunctional uterine bleeding due to anovulation (irregular, prolonged cycles) is the most common reason for abnormal uterine bleeding in the adolescent. However, those who present at or close to menarche with heavy vaginal bleeding, especially if they present to the emergency department, are more likely to have a bleeding disorder. Among those admitted for acute menorrhagia to the Hospital for Sick Children, 20% had a primary coagulation disorder. The most common hematological diagnosis was idiopathic thrombocytopenic purpura followed by von Willebrand disease. It is, of course, always important to exclude pregnancy as well as thyroid dysfunction. Mild bleeding disorders do not tend to present with the classical picture of easy bruising and epistaxis; however, menstruation may unmask these milder forms. If there is any question of a bleeding disorder, then further investigation is warranted because it is well recognized that older women presenting with menorrhagia, as a result of underlying mild bleeding disorders such as von Willebrand disease, are often underdiagnosed. The diagnosis of a bleeding disorder can be difficult because the screening tests, such as PTT and bleeding time, are often normal in von Willebrand disease type 1, therefore, a high index of suspicion based on history is needed. If the diagnosis of a bleeding disorder is made and the primary symptom is heavy vaginal bleeding, then medical management is the mainstay of therapy. Oral contraceptives (cyclic or continuous) and medroxyprogesterone acetate (Depo-Provera, Pharmacia, Mississauga) control menorrhagia in the majority of these patients without the need for additional therapy. Hematological agents that can be considered are DDAVP (nasal or oral) which induces the release of vWf from endothelial cells, or oral antifibrinolytic agents such as tranexamic acid or epsilon aminocaproic acid, which can be given during menstruation. The patient should be instructed to avoid medications with antiplatelet activity such as acetylsalicylic acid and to a lesser extent nonsteroidal anti-inflammatory drugs. Rarely, gonadotropin-releasing hormone analogues or surgery are necessary as last resorts to preserve fertility. The prognosis for future reproduction for these young women is excellent if managed by a team approach in a hospital prepared to deliver pregnant women with bleeding disorders. Menorrhagia at or close to menarche is not uncommonly caused by von Willebrand disease (especially in adolescents presenting to the emergency department with this problem). The PTT and bleeding time can be elevated but are more often normal, and specific testing (vWf antigen and ristocetin cofactor) is required to make the diagnosis. If the primary symptom is vaginal bleeding, then oral contraceptives or Depo-Provera are the mainstays of therapy.

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