Postpartum-acquired hemophilia A presenting as a recurrent rectus sheath hematoma following cesarean section: a case report

Acquired hemophilia A is a rare autoimmune bleeding disorder caused by autoantibodies against factor VIII. Peripartum cases account for approximately 7–11% of the total, and untreated mortality reaches 10–20%. Postpartum-acquired hemophilia A typically presents with subcutaneous bruising, mucosal bleeding, intramuscular hematomas, or retroperitoneal bleeding; presentation as an isolated rectus sheath hematoma masquerading as a surgical complication of cesarean section is uncommon and may delay diagnosis. A 30-year-old Sri Lankan (South Asian) para 2 woman presented 17 days after an uncomplicated elective second cesarean section with severe abdominal pain and a sub-rectus sheath hematoma. Her first pregnancy had been complicated by placental abruption at 32 weeks, after which antiphospholipid syndrome screening had been advised but not undertaken. She underwent two surgical evacuations of recurrent hematomas without a discrete bleeding source, and developed multifocal bleeding including hematuria and wound oozing. Coagulation studies showed an isolated prolongation of the activated partial thromboplastin time (APTT), and inhibitor screening was positive for an acquired, time-dependent inhibitor consistent with a factor VIII inhibitor. Antinuclear antibody was positive (nuclear speckled pattern). Management combined recombinant activated factor VII (rFVIIa), tranexamic acid, blood product support, and immunosuppression with oral prednisone 1 mg/kg/day. The course was complicated by a polymicrobial wound infection ( Pseudomonas and Klebsiella species) treated with meropenem and clindamycin. Marked clinical improvement occurred within 48 hours of immunosuppression, and she was discharged on day 11. Postpartum-acquired hemophilia A should be considered in any postpartum woman with unexplained spontaneous soft-tissue or mucosal bleeding when the APTT is prolonged in isolation and surgical exploration finds no discrete bleeding source. Inhibitor screening is the pivotal diagnostic investigation. The case is particularly relevant to clinicians in low- and middle-income countries, where multidisciplinary clinical reasoning combined with widely available therapies can deliver excellent outcomes when the diagnosis is recognized early.