Pelvic surgery in a child with hemophilia C: a rare disease, a real challenge, a simple solution

SUMMARY Coagulation factor XI deficiency, also called hemophilia C, is the least common type of hemophilia in Norway. It differs from hemophilia A and B in its unpredictable clinical pattern. Patients often have no spontaneous bleeding tendency, but they can have serious and life-threatening bleedings when suffering trauma or undergoing surgical procedures. We report the case of a child with a hitherto undiagnosed factor XI deficiency. She had previously experienced excessive bleeding and required transfusion of saline-adenine-glucose erythrocytes after minor surgery. The patient was planned to a major surgical procedure with partial pelvic resection (hemipelvectomy) because of an Ewing sarcoma. Routine preoperative coagulation tests showed a moderately elevated international normalized ratio and a substantially prolonged activated partial thromboplastin time, which were due to a factor XI level at 0.2% of normal. Perioperative management of the patient was planned in collaboration with a coagulation specialist. In addition to replacement of erythrocytes, two different mechanisms were targeted for correcting the hemostatic defect. Substitution of factor XI was done by infusion of plasma, and inhibition of fibrinolysis was achieved by systemic and local administration of tranexamic acid. Surgery was then performed without excessive bleeding, and the patient did not require postoperative blood transfusions.