A 24-year-old woman was being evaluated by her gynecologist for menorrhagia. She gave a history of excessive menses since the age of 12. A CBC revealed a microcytic anemia and she began a course of ferrous sulfate therapy. Three months later, she had a follow-up visit with her gynecologist, and although her anemia was being corrected, she still complained of excessive menses. Her physician recommended her for a hematology consult. When asked about her family history, she revealed that her brother and mother had recurrent epistaxis and that her first cousin had a postpartum hemorrhage. The consulting physician ordered a CBC, PT, PTT, platelet aggregation studies, and bleeding time. Based upon this patient's history, what is the most likely outcome of this testing and what additional tests are to be considered?
This patient gives a strong family history of mucosal bleeding. Although no member of her family has received a diagnosis of a bleeding disorder, it seems likely that she and some of them may have von Willebrand's disease, an autosomal dominant disorder. The patient's CBC and platelet count is normal; however, the PTT is slightly prolonged at 42 seconds. Factor assays for factor VIII and factor IX should be considered. Aggregation studies with collagen, ADP, and epinephrine were normal. Ristocetin aggregation was absent and the bleeding time test was abnormal with a result of 12 minutes (reference range, 3 to 9 minutes). A preliminary diagnosis of type 1 von Willebrand's disease was made pending the result of the vWF: AG by immunoassay. The hematologist recommended contraceptives as a way to control the patient's menstrual bleeding, and the patient was counseled on therapy alternatives such as DDAVP should she need dental extractions or minor surgery.
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