A 22-year-old woman comes to the emergency department due to heavy vaginal bleeding. The patient has always had heavy menses, but this time the bleeding has not stopped since it started 5 days ago. She has had to change her pad every 1-2 hours. The patient is recovering from an upper respiratory tract infection. Her medications include ibuprofen for headaches and iron supplementation for iron deficiency anemia. She has no other significant medical history. Her mother had heavy menstrual periods and frequent nosebleeds.
Vital signs are within normal limits. Bimanual examination reveals no palpable abnormalities.
Laboratory results are as follows:
Which of the following is the best next step in management of this patient?

Question

A 22-year-old woman comes to the emergency department due to heavy vaginal bleeding.  The patient has always had heavy menses, but this time the bleeding has not stopped since it started 5 days ago.  She has had to change her pad every 1-2 hours.  The patient is recovering from an upper respiratory tract infection.  Her medications include ibuprofen for headaches and iron supplementation for iron deficiency anemia.  She has no other significant medical history.  Her mother had heavy menstrual periods and frequent nosebleeds.
Vital signs are within normal limits.  Bimanual examination reveals no palpable abnormalities.
Laboratory results are as follows:
 Which of the following is the best next step in management of this patient?

Quizplus · Accepted Answer

11ec193a_9619_bf29_853d_01bc030988ea_MD0002_00 This patient has heavy menstrual bleeding (eg, mucocutaneous bleeding), normal coagulation studies and platelet counts, and a family history suggestive of bleeding diathesis (mother with heavy menses and nosebleeds), consistent with von Willebrand disease (an autosomal dominant disorder). In such patients, use of nonsteroidal anti-inflammatory drugs (eg, ibuprofen) may precipitate bleeding. Von Willebrand factor (vWF) binds to platelets and subendothelial collagen receptors at exposed injury sites. It also binds to adjacent platelets and is a carrier protein for factor VIII (FVIII), which has reduced concentration and half-life in the unbound state. Therefore, a deficiency of vWF typically prolongs activated PTT (aPTT). However, in some patients, mild to moderate vWF deficiency may not cause elevated aPTT levels. aPTT can also be falsely normal due to increased vWF (acute phase reactant) and FVIII levels during stress, pregnancy, oral contraceptive use, or liver disease. These tests should be repeated after resolution of these conditions. aPTT Patients with suspected von Willebrand disease require additional testing (Choice D). Screening tests include plasma vWF antigen, plasma vWF activity (ristocetin cofactor activity), and FVIII activity. Treatment includes desmopressin (DDAVP), which causes release of vWF and FVIII from endothelial cells. (Choice A) Mild factor IX deficiency (hemophilia B) can also present with bleeding and normal coagulation studies (rather than prolonged PTT). However, hemophilia (A and B) typically presents with recurrent hemarthroses (ie, joint bleeding) and soft-tissue hematoma in addition to mucocutaneous bleeding. Hemophilia has an X-linked inheritance pattern; therefore, most patients are men. (Choice B) Fibrinogen is required to produce fibrin clotting at the end of the coagulation pathway. Therefore, fibrinogen disorders will result in prolonged PT, aPTT, and thrombin time. (Choice C) Mixing studies are performed in patients with coagulation defects (prolonged prothrombin time and/or PTT) and bleeding diathesis. The coagulation studies correct with plasma mixing in factor deficiencies; an inhibitor (or presence of lupus anticoagulant) will cause persistently prolonged PTT or prothrombin time with plasma mixing. Educational objective: Von Willebrand disease is a common bleeding disorder that can present with mucocutaneous bleeding (eg, epistaxis, heavy menses) and normal platelet counts and coagulation studies. Diagnosis requires plasma von Willebrand factor antigen and activity assays. Treatment is with desmopressin (DDAVP), which causes release of von Willebrand factor and factor VIII from endothelial cells. __________ References: Von Willebrand disease: pathogenesis and management. (http://www.ncbi.nlm.nih.gov/pubmed/23452742) High resolution multimer analysis and the PFA-100 platelet function analyser can detect von Willebrand disease type 2A without a pathological ratio of ristocetin cofactor activity and von Willebrand antigen level. (http://www.ncbi.nlm.nih.gov/pubmed/23289190)

A 22-Year-Old Woman Comes to the Emergency Department Due to Heavy