INTRODUCTIONFactor XI deficiency (FXI) is associated with significant bleeding in the setting of trauma and surgery. Red blood cell (RBC) allo‐antibodies present the unique challenge that RBC units may be difficult to obtain when needed. We present a patient with Factor XI deficiency and multiple RBC allo‐antibodies requiring repeat aortic root replacement and discuss the perioperative management of these patients.PATIENT PROFILEA 62‐year‐old male with severe FXI deficiency, Hepatitis C virus (HCV) infection, and RBC allo‐antibodies requiring repeat sternotomy for repair of an ascending aorta aneurysm and aortic valve replacement.The patient was diagnosed with severe FXI deficiency (Factor XI level <1%, baseline partial thromboplastin time [PTT] 53 s) in 1965 at the age of 10 when he presented with a right periorbital hematoma after a fall. He was treated successfully with fresh frozen plasma (FFP) infusions.The patient had no serious hematologic complications until 6 years before his current presentation, when head imaging performed as part of a neurocognitive evaluation revealed a spontaneous subdural hematoma. He underwent urgent craniotomy with subdural evacuation after pretreatment with FFP. One year before this presentation, after a routine outpatient hemorrhoidectomy, the patient developed persistent lower gastrointestinal bleeding. Six days after the procedure, he was evaluated in
Journal of Cardiac Surgery – Wiley
Published: Jan 1, 2018
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