Moderate hemophilia C in pregnancy: a rare case of transfusion-related complications and life-saving off-label use of recombinant factor VIIa

Hemophilia C (factor XI deficiency) is a rare bleeding disorder inherited in an autosomal recessive pattern. We present a case of a 32-year-old pregnant woman diagnosed with moderate hemophilia C who developed life-threatening complications after receiving fresh frozen plasma (FFP) for peripartum prophylaxis. After an initial mild allergic reaction to a single unit of FFP, she subsequently received a high-volume FFP transfusion for peripartum prophylaxis and developed acute respiratory deterioration requiring urgent evaluation for TACO versus TRALI. While awaiting the results of the differential diagnosis, she developed massive hemorrhage from the postoperative wound. Administration of further FFP doses was considered to carry a high risk of complications. Therefore, off-label recombinant factor VIIa (rFVIIa) was administered. This case highlights the clinical challenges and limited therapeutic options for pregnant patients with factor XI deficiency and supports the potential role of rFVIIa as a life-saving intervention.