Department of Orthopedic Surgery, Naha City Hospital, Okinawa, Japan.
Department of Anesthesiology, Naha City Hospital, Okinawa, Japan.
Spine (Phila Pa 1976). 2021 Mar 1;46(5):E349-E352. doi: 10.1097/BRS.0000000000003805.
Case report.
To summarize the clinical manifestations and treatment of Factor XI deficiency in a patient with cervical spondylotic myelopathy.
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.
A case was investigated retrospectively and the relevant literature was reviewed.
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.
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.
病例报告。
总结凝血因子 XI 缺乏症合并颈椎病患者的临床表现和治疗方法。
凝血因子 XI 缺乏症是一种罕见的遗传性出血性疾病，由凝血因子 XI 水平降低和活性不足引起。据报道，这种疾病与创伤和手术无关，但与 FXI 水平无关，会导致明显出血。这是首例凝血因子 XI 缺乏症合并颈椎病患者。
回顾性调查病例，并复习相关文献。
一名 66 岁男性，手指灵活性丧失和步态障碍病史 2 个月，就诊于我科。他没有出血或凝血障碍病史，家族中也没有相关病史。颈椎磁共振成像（MRI）显示 C3/4 至 C5/6 椎管狭窄，C3/4 脊髓 T2 加权像（T2WI）上呈髓内高信号。术前检查无异常发现，但激活部分凝血活酶时间（APTT）严重延长至 139.8 秒。凝血因子活性测定显示凝血因子 XI 严重缺乏（<0.1%）。根据血液科医生的建议，在手术前一天输注了 4 单位新鲜冷冻血浆（FFP），手术当天清晨 APTT 测定值为 70.5 秒。手术期间又输注了 4 单位 FFP，APTT 为 60 秒。术后过程顺利，患者于术后第 14 天出院。
凝血因子 XI 缺乏症患者在创伤或手术后可能会发生过度出血。对于 APTT 延长的患者，应进行术前检查，直至确诊。在诊断为凝血因子 XI 缺乏症之前，应注意精细的围手术期出血管理，包括脊髓手术后的血肿。
5。
