ANSWER
Due to increased vaginal bleeding, a 29-year-old female (G1P1) is readmitted two weeks after vaginal delivery. She claims the bleeding started on the tenth day after delivery and has worsened with each passing day. The delivery was straightforward, with minimal blood loss, and the patient received no epidural anesthesia. She has been taking 200 mg of ibuprofen every day since giving birth. The patient has a history of iron deficiency anemia caused by menorrhagia. She was adopted and did not know her family history.
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The patient has received three units of blood since his admission. The obstetrics team has ruled out the cause of the bleeding as retained placental tissue and uterine atony. Her lab results are as follows:
2.1 g/L hemoglobin (13.7-17.5 g/dL)
Platelets 110 k/L (range: 161-347 k/L)
Partial activation 40-second thromboplastin time (25-37 sec)
Prothrombin time: 15 seconds (11-17 sec)
Fibrinogen (177-466 mg/dL) 200 mg/dL
What hematologic disorder is most likely causing her bleeding?
Platelet dysfunction caused by NSAIDs
Acquired factor VIII inhibitors cause Von Willebrand disease
Intravascular coagulation that has spread throughout the body
Hemolytic anemia caused by microangiopathy
Answer
Explanation of von Willebrand disease
This patient has secondary postpartum hemorrhage and a clinical and laboratory history consistent with von Willebrand disease (vWD). Due to increased vaginal bleeding, a 29-year-old female (G1P1) is readmitted two weeks after vaginal delivery. She claims the bleeding started on the tenth day after delivery and has worsened with each passing day. The delivery was straightforward, with minimal blood loss, and the patient received no epidural anesthesia. She has been taking 200 mg of ibuprofen every day since giving birth. The patient has a history of iron deficiency anemia caused by menorrhagia. She was adopted and did not know her family history.
The patient has received three units of blood since his admission. The obstetrics team has ruled out the cause of the bleeding as retained placental tissue and uterine atony. Her lab results are as follows:
2.1 g/L hemoglobin (13.7-17.5 g/dL)
Platelets 110 k/L (range: 161-347 k/L)
Partial activation 40-second thromboplastin time (25-37 sec)
Prothrombin time: 15 seconds (11-17 sec)
Fibrinogen (177-466 mg/dL) 200 mg/dL
What hematologic disorder is most likely causing her bleeding?
Platelet dysfunction caused by NSAIDs
Von Willebrand disease is caused by acquired factor VIII inhibitors
Intravascular coagulation that has spread throughout the body
Hemolytic anemia caused by microangiopathy
Answer
Explanation of von Willebrand disease
This patient has secondary postpartum hemorrhage and a clinical and laboratory history consistent with von Willebrand disease (vWD). Peripartum bleeding is associated with vWD in 20% of cases, and severe bleeding occurs in 75% of women with moderate to severe vWD. Any vWD can cause bleeding. The peripartum period may be the first manifestation of vWD in women with mild forms of the disease. 1 In addition, because peripartum bleeding unrelated to a bleeding disorder is expected, the diagnosis of an underlying bleeding diathesis may be overlooked.
Both factor VIII and von Willebrand factor (vWF) levels rise during pregnancy, with peaks at 29 to 32 weeks and 35 weeks, respectively.
2 Within the first few weeks after delivery, vWF levels may precipitate, resulting in delayed uterine bleeding. 3 Monitoring vWF levels during pregnancy and for three to four weeks postpartum to ensure the return to baseline is part of managing women with known vWD. The severity of the disease and the specific type of vWD determine the prevention and treatment of vWD during pregnancy.
A severe but rare cause of secondary postpartum hemorrhage is acquired factor VIII inhibitors.
4 When a factor VIII inhibitor is present, the activated partial thromboplastin time (aPTT) is typically significantly prolonged. Diffuse intravascular coagulation is a major cause of postpartum hemorrhage. Significant aPTT and PT prolongations and low fibrinogen levels are required to make the diagnosis. Microangiopathic hemolytic anemia, associated with low hemoglobin and platelets, can occur during pregnancy. This diagnosis, however, results in microvascular thrombosis rather than hemorrhage. Peripartum bleeding is associated with vWD in 20% of cases, and severe bleeding occurs in 75% of women with moderate to severe vWD. Any vWD can cause bleeding. The peripartum period may be the first manifestation of vWD in women with mild forms of the disease. 1 In addition, because peripartum bleeding unrelated to a bleeding disorder is expected, the diagnosis of an underlying bleeding diathesis may be overlooked.
Both factor VIII and von Willebrand factor (vWF) levels rise during pregnancy, with peaks at 29 to 32 weeks and 35 weeks, respectively.
2 Within the first few weeks after delivery, vWF levels may precipitate, resulting in delayed uterine bleeding. 3 Monitoring vWF levels during pregnancy and for three to four weeks postpartum to ensure a return to baseline is part of managing women with known vWD. The severity of the disease and the specific type of vWD determine the prevention and treatment of vWD during pregnancy.
A severe but rare cause of secondary postpartum hemorrhage is acquired factor VIII inhibitors.
4 When a factor VIII inhibitor is present, the activated partial thromboplastin time (aPTT) is typically significantly prolonged. Diffuse intravascular coagulation is a major cause of postpartum hemorrhage. Significant aPTT and PT prolongations and low fibrinogen levels are required to make the diagnosis. Microangiopathic hemolytic anemia, associated with low hemoglobin and platelets, can occur during pregnancy. This diagnosis, however, results in microvascular thrombosis rather than hemorrhage.
QUESTION
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Hypothetical case of hemorrhage