Factor XI Deficiency in a Patient with Cervical Spondylotic Myelopathy

Abstract

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

In Brief

We summarize the clinical manifestations and treatment of Factor XI deficiency in a patient with cervical spondylotic myelopathy. The patients with severe FXI deficiency undergoing surgical procedure will require prophylactic treatment, even if they have a negative history of excessive bleeding.