Acquired Postpartum Hemophilia A Presentation of Severe Hematuria: A Case Report

Abstract

BACKGROUND: Acquired hemophilia A (AHA) is a rare, severe bleeding disorder caused by the development of autoantibodies against FVIII that may be idiopathic or secondary to medical conditions. Postpartum AHA can occur as early as 1–4 months after delivery or as late as 1-year postpartum. CASE REPORT: A 20-year-old female presented with vaginal bleeding 20 days after delivery, then ecchymotic patches developed 2 months later, followed by hematuria 2 months after that. Laboratory investigation revealed isolated, prolonged partial thromboplastin time that was not corrected by mixing 50:50 with fresh normal plasma. FVIII activity was markedly deficient, with a high titer of immediate-acting FVIII inhibitor antibodies. CONCLUSION: The case was diagnosed as postpartum AHA with a good response to the combined steroids and cyclophosphamide treatment.