Two aspects of this patient's presentation were not consistent with a congenital deficiency of an intrinsic pathway factor. First, bleeding began late in life rather than during infancy or childhood. Secondly, mixing studies performed in the laboratory were abnormal. This time and temperature dependency is classic for specific inhibitors of clotting factors. In addition, patients with lupus anticoagulants do not bleed or bruise excessively, but instead are at increased risk for thromboembolic complications.
Management involves treatment of acute bleeding complications and immunosuppression therapy.
The primary use of FIX complex concentrates is to replace FIX in Hemophilia B patients. A few days later the patient felt a sudden ache in her left groin while climbing stairs. The pain steadily increased and was aggravated by flexion of her left leg. The symptoms were classic for an iliopsoas hematoma which was confirmed on CT scan, and she was hospitalized. An infusion of a purposely activated FIX complex concentrate very eight hours was begun.