Double Trouble: Combined Prekallikrein and IgA Deficiencies in a Patient Undergoing Orthotopic Heart Transplantation-A Case Report.

Prekallikrein (PK) and selective IgA deficiencies are rare, and their coexistence in a cardiac transplant patient presents unique challenges. These disorders affect anticoagulation monitoring and transfusion safety, necessitating tailored perioperative strategies. We present the case of a 58-year-old female with both PK and IgA deficiency who underwent orthotopic heart transplantation (OHT), complicated by significant postoperative bleeding requiring re-exploration. A multidisciplinary plan included FFP-based IgA desensitization and correction of elevated ACT from PK deficiency. Anti-Xa levels were used to confirm anticoagulation. The patient tolerated unwashed blood products without anaphylaxis and recovered uneventfully. This is the first known report of combined PK and IgA deficiencies in cardiac surgery. It highlights a successful strategy involving perioperative FFP administration for both desensitization and ACT normalization. These measures ensured safe anticoagulation and transfusion in a high-risk setting.