Factor XI Deficiency in a Patient with Cervical Spondylotic Myelopathy.

STUDY DESIGN: Case report. OBJECTIVE: To summarize the clinical manifestations and treatment of Factor XI deficiency in a patient with cervical spondylotic myelopathy. SUMMARY OF BACKGROUND DATA: Factor XI deficiency is a rare genetic bleeding disorder caused by reduced levels and insufficient activity of a coagulation factor XI. It is claimed to be associated with prominent bleeding in case of trauma and surgery irrelevant to the FXI level. This is the first ever case of a patient with factor XI deficiency with cervical spondylotic myelopathy. METHODS: A case was investigated retrospectively and the relevant literature was reviewed. RESULTS: A 66-year-old man with a 2-months history of lack of finger dexterity and gait disturbance was referred to our department. He did not have a history of bleeding or coagulation disorder nor did his family. Magnetic resonance imaging (MRI) of the cervical spine revealed spinal canal stenosis at C3/4 to C5/6 and intramedullary hyperintensity at C3/4 on the :T2 weighted image (T2WI). Preoperative examination revealed no abnormal findings but a severe prolonged activated partial-thromboplastin time (APTT) of 139.8 seconds. Coagulation factor activity assay revealed severe deficiency of factor XI (<0.1%). In accordance with hematologist's recommendation, four units of fresh frozen plasma (FFP) were transfused on the day before surgery and APTT assayed early morning on the day of surgery was 70.5 seconds. An additional four units of FFP were transfused during the surgery and APTT was 60 seconds. The postoperative course was uneventful and the patient was discharged on the postoperative day 14. CONCLUSION: Factor XI deficiency patients may develop excessive bleeding after trauma or surgery. Preoperative examination with prolonged APTT should be pursued until a diagnosis of is made. Under diagnosis of Factor XI deficiency, meticulous attentions are required for perioperative bleeding management including postoperative hematoma in spinal surgery.Level of Evidence: 5.

Pretruncal Nonaneurysmal Subarachnoid Hemorrhage with Underlying Hemophilia C.

BACKGROUND: Pretruncal nonaneurysmal subarachnoid hemorrhage (PNSAH) accounts for 15%-12% of all case of subarachnoid hemorrhage. Its precise etiology is not yet established. Multiple theories and risk factors have been investigated to address the possible cause of this type of hemorrhage including basilar tip dissecting aneurysms, high spinal arteriovenous fistula, venous stenosis/hypertension or venous bleeding. Hereditary coagulopathies and hemophilias have rarely been reported in the literature as a potential cause of PNSAH. CASE DESCRIPTION: Here, we reported a rare case of PNSAH with negative angiogram and magnetic resonance imaging who was also found to have hemophilia C (factor XI deficiency) confirmed by laboratory investigation. We also included a literature review of hereditary coagulopathies and their role as a possible cause of PNSAH. CONCLUSIONS: Detailed medical history and physical examination of patients with PNSAH may lead to further hematologic evaluation for this group of patients, as in this case, and may reveal more cases of mild coagulopathy that require treatment.

A surgical case of cerebral hemorrhage in a patient with factor XI deficiency.

A 63-year-old man suddenly presented with right hemiplegia and was taken to our hospital. Computed tomography (CT) scan revealed subcortical hemorrhage of the left parietal lobe. He had no medical history except hypertension; thus, it initially appeared to be a typical hypertensive hemorrhage. However, blood analysis showed an abnormally elevated activated partial thromboplastin time. One hour after admission, his Glasgow Coma Scale fell from 14 to 11. We performed an echo-guided stereotaxic removal of the hematoma. He improved immediately and was diagnosed with congenital factor XI (FXI) deficiency a few days after surgery. FXI deficiency, described as hemophilic syndrome C, rarely manifests as spontaneous bleeding, but surgical intervention has been known to manifest as bleeding. This case highlights the importance of evaluation of coagulopathies in patients with intracerebral hemorrhage before surgery, and, in cases wherein blood analysis results suggest coexisting coagulation disorders, less invasive surgical methods would likely lead to good outcomes.