Managing Relapsed Acquired Hemophilia A During Pregnancy: A Case Report on Peripartum Hemostasis

Acquired Hemophilia A (AHA) represents a rare and complex bleeding disorder characterized by the production of autoantibodies or inhibitors specifically targeting coagulation factor VIII. This condition poses significant clinical challenges, particularly when it manifests during pregnancy, a scenario where definitive management guidelines remain scarce due to the rarity of prenatal presentations. This case report details the successful peripartum management of a 30-year-old patient experiencing a relapse of AHA while pregnant, highlighting the critical role of bypassing agents in preventing severe postpartum hemorrhage and underscoring the necessity of stringent monitoring for the newborn.

The patient, a 30-year-old woman, was initially diagnosed with AHA after presenting with gross bleeding during a laparoscopic surgery for corpus luteum rupture in July 2018. Diagnostic workup revealed an isolated and obvious prolongation of the Activated Partial Thromboplastin Time (APTT) to 119 seconds, while her Prothrombin Time (PT) and platelet count remained within normal reference ranges. A crucial 1:1 incubated APTT mixing study, conducted over a two-hour period at 37 degrees Celsius, confirmed the presence of a time-dependent circulating coagulation inhibitor. Further specific testing demonstrated that the FVIII activity was less than 1%, and the FVIII inhibitor titer was measured at 992 BU/mL using the Bethesda assay. Comprehensive serological screening for common secondary etiologies, including connective-tissue autoimmune diseases (ANA, anti-dsDNA, anti-ENA spectrum, rheumatoid factor), was negative. Furthermore, tumor markers and extensive imaging ruled out underlying malignancy, and no history of drug exposure or active bacterial infection was noted, suggesting a primary autoimmune etiology.

Initial therapeutic efforts to manage the relapsed AHA involved a combination of prednisone (40 mg, 1 mg/kg, daily) and CTX (50/100 mg, every other day). However, after one month, when prednisone was reduced to 35 mg daily, no decline in the FVIII inhibitor level or rise in FVIII activity was observed, strongly suggesting a poor response to this initial regimen. Due to high Hepatitis B virus DNA levels (2.8 x 10^8 copies/mL) and the lack of response, Rituximab was initially held. The immunosuppressive therapy was subsequently switched to azathioprine (AZA) at 100 mg daily, which also yielded no benefit. The treatment was then escalated to Cyclosporin at 75 mg daily, but the response remained unsatisfactory. The clinical course required further intervention, leading to the administration of Rituximab (375 mg/m2 weekly for four cycles). Following this intensive treatment, a significant improvement was noted: FVIII activity gradually increased to 33%, and the FVIII inhibitor became undetectable six months after the administration of Rituximab, indicating a state of partial remission. Cyclosporin was subsequently selected as the maintenance therapy, alongside antiviral therapy involving entecavir and later tenofovir, which successfully reduced the HBV DNA levels.

The patient’s life changed in July 2020 when she experienced an unintended pregnancy. Despite a thorough discussion regarding the inherent risks associated with continued gestation in a patient with refractory AHA, she chose to proceed with the pregnancy while maintaining her maintenance dose of Cyclosporin 75 mg bid. Throughout the pregnancy, regular monitoring revealed a gradual, yet concerning, increase in FVIII inhibitor titers, rising from zero to 51.2 BU/mL, concurrent with a decrease in FVIII activity from 30% to 3.4% before delivery. Crucially, the pregnancy progressed without any significant bleeding episodes, which the authors suggest may be partially attributable to a hypercoagulation propensity in the setting of the disease. At 37 weeks and 4 days, a cesarean section was performed. To mitigate the risk of severe postpartum hemorrhage, the procedure was preceded by an immediate intravenous injection of recombinant activated Factor VII (rFVIIa) at a dose of 4 mg (88.8 μg/kg).

Postoperative complications arose, with active bleeding noted at the incision site and cervix. The hemorrhage was successfully controlled through the combined use of immediate uterine artery embolization and uterine tamponade. The estimated operative blood loss was substantial, totaling 900 mL, which caused the patient’s hemoglobin levels to drop from 110 g/L to 85 g/L. Following transfusion with two units of red blood cell concentrates, 400 mL of Fresh Frozen Plasma (FFP) was also administered due to intraoperative evidence suggesting mild global coagulation factor consumption, observed after the application of embolization and tamponade. The bypassing agent, rFVIIa, was administered repeatedly, initially four hours after surgery and subsequently every eight hours. Due to financial constraints, the hemostatic regimen was later switched to Prothrombin Complex Concentrate (PCC) at a dosage of 900 U every 12 hours intravenously. The PCC treatment was discontinued on postoperative day six as the patient’s condition stabilized.

The newborn was also monitored closely for signs of maternal-derived inhibitor transfer. Immediate umbilical cord blood testing revealed a prolonged APTT of 120 seconds, with FVIII activity at 1.2% and an FVIII inhibitor titer of 11.2 BU/mL, confirming the presence of a maternal-derived FVIII inhibitor. Despite the presence of these inhibitors, cranial and abdominal ultrasounds showed no signs of bleeding. No specific hemostatic treatment was administered to the infant. Close monitoring demonstrated that the newborn’s FVIII inhibitors spontaneously disappeared by day 14, and the child experienced no bleeding episodes. This observation aligns with previous literature suggesting that inhibitors may resolve spontaneously without hemorrhagic events, although severe intracranial hemorrhage has been reported in other cases of transplacental transfer.

The long-term follow-up of the mother showed that after discharge, she continued her Cyclosporin regimen, and her FVIII activity increased to 2.7% while her FVIII inhibitor level rose to 52 BU/mL. Attempts to switch to Sirolimus were unsuccessful. Although Rituximab was administered again, remission was not achieved. At the latest follow-up, when the child was five years old, he demonstrated age-appropriate growth and had experienced no bleeding events since the spontaneous clearance of the transplacentally transferred FVIII inhibitor on postnatal day 14. This case underscores the critical need for thorough pre-conception counseling for AHA patients who have not achieved complete disease remission.

In conclusion, this case report provides valuable insights into the successful peripartum management of a patient with a relapsed AHA during pregnancy. The use of bypassing agents, such as rFVIIa and PCC, proved to be a safe and effective strategy for preventing severe postpartum hemorrhage. Furthermore, the observation that immunosuppressive therapies administered both during pregnancy and the postpartum period failed to eliminate the FVIII inhibitors highlights a major clinical limitation. While the management of the mother’s bleeding was successful, the persistence of the FVIII inhibitor in the infant, despite its spontaneous clearance, emphasizes the necessity of close monitoring for newborns exposed to maternal FVIII inhibitors, even in the absence of immediate clinical bleeding.

Subject of Research: Clinical management of Acquired Hemophilia A during pregnancy and peripartum hemorrhage prevention.

Article Title: Case Report: Successful peripartum management of acquired hemophilia A relapse during pregnancy

Article References: Chen, H., Yu, X., & Zhu, T. (2026). Case Report: Successful peripartum management of acquired hemophilia A relapse during pregnancy. Frontiers in Medicine, 13, Article 1963805. https://doi.org/10.3389/fmed.2026.1963805

Image Credits: AI Generated

DOI: 10.3389/fmed.2026.1963805

Keywords: Acquired Hemophilia A, AHA, Pregnancy Complications, Hemostasis, Immunosuppression, Bypassing Agents, Postpartum Hemorrhage, Case Report, Factor VIII Inhibitors, Case, Report, Successful