A 34-year-old woman, gravida 1 para 1, comes to the emergency department due to sudden-onset heavy vaginal bleeding. For the last 4 hours, she has felt weak and has had heavy bleeding that has soaked 2 pads per hour. She has no associated fever, nausea, vomiting, or abdominal pain. Two weeks ago, the patient had a forceps-assisted vaginal delivery complicated by postpartum bleeding from a large vaginal laceration that required extensive repair. She had a blood transfusion on postpartum day 1 and was discharged home on postpartum day 3. Temperature is 36.7 C (98 F), blood pressure is 100/52 mm Hg, and pulse is 98/min. The abdomen is soft and nontender. Pelvic examination shows a well-approximated vaginal laceration. The cervix is slightly dilated and has active bleeding from the os, but no cervical or additional vaginal lacerations are present. The uterus is small, firm, mobile, and nontender. Laboratory results are as follows: Pelvic ultrasound shows no endometrial masses or increased vascularity of the uterus. Which of the following is the most likely cause of this patient's postpartum hemorrhage?

Question

A 34-year-old woman, gravida 1 para 1, comes to the emergency department due to sudden-onset heavy vaginal bleeding.  For the last 4 hours, she has felt weak and has had heavy bleeding that has soaked 2 pads per hour.  She has no associated fever, nausea, vomiting, or abdominal pain.  Two weeks ago, the patient had a forceps-assisted vaginal delivery complicated by postpartum bleeding from a large vaginal laceration that required extensive repair.  She had a blood transfusion on postpartum day 1 and was discharged home on postpartum day 3.  Temperature is 36.7 C (98 F), blood pressure is 100/52 mm Hg, and pulse is 98/min.  The abdomen is soft and nontender.  Pelvic examination shows a well-approximated vaginal laceration.  The cervix is slightly dilated and has active bleeding from the os, but no cervical or additional vaginal lacerations are present.  The uterus is small, firm, mobile, and nontender.  Laboratory results are as follows:  Pelvic ultrasound shows no endometrial masses or increased vascularity of the uterus.  Which of the following is the most likely cause of this patient's postpartum hemorrhage?

Quizplus · Accepted Answer

Explanation: 11ec25c8_da64_d1e5_bc81_8f5e01849cd4_MD0003_00 This patient with a postpartum hemorrhage and a prolonged bleeding time but otherwise normal coagulation studies likely has von Willebrand disease (VWD). VWD is a common coagulopathy that is due to a quantitative or qualitative von Willebrand Factor (VWF) deficiency. It typically presents with mucocutaneous bleeding (eg, epistaxis, gingival bleeding) and is a common cause of secondary (or late) postpartum hemorrhage. VWF assists in platelet adhesion and aggregation and acts as a carrier protein for factor VIII. Factor VIII is more rapidly degraded when unbound, and VWF deficiency can therefore lead to impairment of the intrinsic coagulation pathway (sometimes leading to a prolonged activated PTT). However, patients can have a normal aPTT for a variety of reasons: platelet adhesion and aggregation intrinsic coagulation pathway Patients with mild to moderate VWF deficiency may have adequate factor VIII levels to maintain aPTT within normal range (most common reason) VWF and factor VIII are acute-phase reactants; stress and inflammation can acutely raise levels VWF synthesis is increased by estrogen and thyroid hormones; pregnancy, oral contraceptives, and thyroid hormone replacement can raise VWF levels and normalize aPTT Platelet count and PT are normal in VWD, but impaired platelet function causes a prolonged bleeding time. Diagnostic studies include VWF antigen level, VWF activity (ristocetin cofactor activity), and factor VIII levels. Desmopressin (DDAVP) potentiates release of VWF from endothelial cells and can be used in management of acute bleeding or for prophylaxis. (Choice A) Gestational trophoblastic disease presents with heavy vaginal bleeding, often after a term delivery. However ultrasound typically shows an enlarged uterus and an intrauterine mass with increased vascularity. (Choice B) Postpartum endometritis can increase postpartum bleeding, but patients typically have fever, uterine tenderness, and a purulent vaginal discharge. This patient is afebrile and has a normal pelvic examination. (Choices C and D) Uterine atony and retained products of conception can cause heavy postpartum bleeding but examination shows an enlarged, boggy uterus. (Choice E) Vaginal hematoma typically presents with hypotension and anemia due to concealed bleeding into the retroperitoneum. In addition, patients have severe vaginal pain and a vaginal mass on examination. This patient has a normal pelvic examination. Educational objective: Patients with von Willebrand disease may present with a postpartum hemorrhage and prolonged bleeding time. Activated PTT may be normal or prolonged, and platelet count and PT are normal. Treatment of acute bleeding includes desmopressin. References: ACOG practice bulletin: clinical management guidelines for obstetrician-gynecologists number 76, October 2006: postpartum hemorrhage. (http://www.ncbi.nlm.nih.gov/pubmed/17012482) Committee opinion no.580: von Willebrand disease in women. (http://www.ncbi.nlm.nih.gov/pubmed/24264714) Screening women with menorrhagia for underlying bleeding disorders: the utility of the platelet function analyser and bleeding time. (http://www.ncbi.nlm.nih.gov/pubmed/16128894)

A 34-Year-Old Woman, Gravida 1 Para 1, Comes to the Emergency