A new report presented in the journal Cureus described a case of peripartum hemophilia A in a patient with sickle cell trait. The case report was written by Syed Hamza Bin Waqar, MD, and colleagues from the State University of New York Downstate Medical Center in Brooklyn, New York.
“Pregnancy-associated acquired hemophilia A remains a mystery and has very scarce literature on its etiology, management in terms of bleeding, and hemostasis achievement,” Dr Bin Waqar and colleagues wrote in their report.
The patient was described as being 31 years of age and having sickle cell trait. She received a cesarean section and delivered a healthy baby. However, hematomas formed at the injection site over the triceps and at the site of incision, and she experienced a profuse bleeding episode around the location of intravenous lines. She was treated with 4 units of fresh frozen plasma, tranexamic acid, and misoprostol. However, her bleeding continued. Symptoms like moderate back pain, progressive weakness of the lower extremities, and numbness and tingling also became apparent.
As bleeding persisted, the patient developed hypotension and received fluids. Her hemoglobin level had declined from baseline by 4.5 g/dL, and her partial thromboplastin time (PTT) was 83 seconds, compared with a preoperative PTT of 49 seconds. She was then given an activated prothrombin complex concentrate (aPCC). Computed tomography of the abdomen and magnetic resonance imaging of the lumbar spine showed multiple hematomas.
The patient’s hemoglobin level continued to drop, and she was given recombinant factor VIIa at a dosage of 90 mg/kg twice, an additional infusion of aPCC, and 3 units of red blood cell transfusions. Laboratory results showed the patient had had a factor VIII level under 2% and an inhibitor level of 72 Bethesda units. The totality of her laboratory results suggested a diagnosis of acquired hemophilia A related to factor VIII.
With treatment to limit bleeding and eliminate inhibitors, the patient’s condition improved, and she received aminocaproic acid on discharge, which was 10 days following delivery. However, she began to bleed from her surgical incision and returned to the emergency department 2 days after discharge. She was then given 4 cycles of rituximab (375 mg/m2 per week) and prednisone (1 mg/kg), with regular monitoring of PTT inhibitors.
She completed the course of rituximab, but her inhibitor level remained elevated at 96 Bethesda units, with factor VIII levels of 1%-4%. Cyclophosphamide (1.5 mg/kg) was then begun, while prednisone was continued. Results from a 2-week follow-up showed improvement, with her inhibitor level at 2 Bethesda units and a factor VIII level of 76%, and she has since been managed at a hematology clinic.
Dr Bin Waqar and colleagues concluded that acquired hemophilia A should be considered as a differential diagnosis in cases of activated PTT derangement during pregnancy. They noted that for patients with pregnancy-associated acquired hemophilia A, the consequences can be disastrous if there are delays in diagnosis, achievement of hemostasis, and initiation of immunosuppression.
Bin Waqar SH, Khoury L, Hussain A, McFarlane IM. Acquired hemophilia A in peripartum period: diagnostic and therapeutic dilemma. Cureus. 2021;13(8):e16803. doi:10.7759/cureus.16803