A 17-year-old girl comes to the office for evaluation of heavy menstrual bleeding. She typically has 4 days of heavy bleeding, often requiring 5 or 6 pads a day. The patient awakens multiple times a night to change her pad and often soaks through her clothing and bed sheets. She has mild cramping on the first 2 days of her menses that is controlled by ibuprofen but has no intermenstrual bleeding or chronic pelvic pain. Menarche was at age 12; menses were irregular the first couple years but are now regular and occur every 26 days. The patient has no chronic medical conditions and has had no surgery. She has a male partner and uses condoms for contraception. Vital signs are normal. BMI is 23 kg/m². On bimanual examination, the uterus is small and mobile; no adnexal mass or tenderness is present. Laboratory results are as follows: Urine pregnancy test is negative. Which of the following is the most likely cause of this patient's heavy menstrual bleeding?

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11ec25c8_d9f3_e7e0_bc81_37f311193c5a_MD0003_00 This patient with heavy, regular menses; anemia; and normal coagulation studies likely has von Willebrand disease (vWD). vWD is a common bleeding disorder that is caused by either a quantitative or qualitative deficiency in von Willebrand factor (vWF). Patients with vWD have impaired platelet adhesion as vWF acts as a bridging glycoprotein between platelets and endothelial factors at sites of vascular injury. vWF also serves as a carrier for factor VIII, preventing its degradation by protein C. Common clinical manifestations of vWD include easy bruising and mucocutaneous bleeding (eg, epistaxis, heavy menstrual bleeding); symptom onset variability (eg, a few years after menarche) is based on disease severity. Patients typically have a prolonged bleeding time; however, platelet count and PT are normal. Patients may have a prolonged activated PTT, but activated PTT can be normal in those with mild disease (as in this patient) or in states of increased vWF synthesis (eg, pregnancy, oral contraceptive use, acute stress, thyroid hormone supplementation). The diagnosis is confirmed by vWF testing (vWF antigen, ristocetin cofactor activity, factor VIII levels). Management, which is rarely needed outside of trauma or surgery, includes desmopressin, vWF replacement therapy, and antifibrinolytics (eg, tranexamic acid). (Choice A) Endometriosis (ectopic endometrial glands) presents with heavy, painful menses and an immobile uterus. This patient has a mobile uterus and menstrual pain that responds to ibuprofen, making this diagnosis unlikely. Endometriosis (Choice B) In the first 1-2 years following menarche, adolescents may have an immature hypothalamic-pituitary-ovarian axis, leading to irregular menses and anovulatory bleeding. Although this patient's menses were initially irregular, they have subsequently normalized. (Choice D) Inherited factor VIII deficiency (ie, hemophilia A) is an X-linked recessive disorder that is much more common in males. It typically manifests with spontaneous hemarthrosis, intramuscular hematomas, and gastrointestinal or genitourinary bleeding. (Choice E) Endometrial polyps (localized hyperplastic growth of endometrial glands) typically present with intermenstrual bleeding, which is not seen in this patient. Endometrial polyps (Choice F) Uterine leiomyomata, the proliferation of myometrial smooth muscle, can cause heavy, regular menses. However, patients typically have a large, irregularly shaped uterus rather than a small, mobile uterus. Uterine leiomyomata Educational objective: Von Willebrand disease is a common bleeding disorder that causes impaired platelet adhesion and is the most common cause of heavy, regular menses in adolescents. Platelet counts and PT are normal, activated PTT may be normal or prolonged. References: ACOG committee opinion number 580: von Willebrand disease in women. (http://www.ncbi.nlm.nih.gov/pubmed/24264714) Practice bulletin no. 128: diagnosis of abnormal uterine bleeding in reproductive-aged women. (http://www.ncbi.nlm.nih.gov/pubmed/22914421)

A 17-Year-Old Girl Comes to the Office for Evaluation of Heavy