获得性凝血因子XI缺乏1例报道。

IF 0.7 4区 医学 Q4 MEDICAL LABORATORY TECHNOLOGY Clinical laboratory Pub Date : 2024-12-01 DOI:10.7754/Clin.Lab.2024.240715
Wenjun Zhu, Shaoli Deng, Jinmi Li
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引用次数: 0

摘要

背景:获得性凝血因子缺乏症是一种由凝血因子抗体产生引起的自身免疫性出血性疾病。获得性凝血因子XI缺乏症的发生率较低且很少报道。病例介绍:我们报告一例患者获得性凝血因子XI缺乏症。患者为24岁女性,入院前表现为皮肤轻度发黄,全身不适,心悸,无明显出血症状,如皮肤和粘膜斑点。她最初被诊断为贫血,因为她的血红蛋白是62克/升。实验室结果显示,活化的部分凝血活素时间(APTT)延长,检测值为83.40秒,无法通过校正实验进行校正;因子XI活性小于0.1%,因子XI抗体阳性,血浆凝血因子XI抑制剂为35.20 BU/mL。最终确诊为获得性凝血因子XI缺乏症,行血浆置换清除抗体,患者病情好转。结论:血浆置换治疗获得性因子XI缺乏症有积极作用。明确的早期诊断和适当的治疗有助于提高患者的治愈率。
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A Rare Case Report of Acquired Coagulation Factor XI Deficiency.

Background: Acquired coagulation factor deficiency is an autoimmune hemorrhagic disease caused by the production of antibodies to coagulation factor. The incidence of acquired coagulation factor XI deficiency is low and rarely reported.

Case presentation: We report a case of a patient with acquired coagulation factor XI deficiency. The patient was a 24-year-old female who presented with mild yellowing of the skin, general malaise, palpitation, and no obvious bleeding symptoms prior to admission, such as skin and mucous membrane petechiae. She was initially diagnosed with anemia because her hemoglobin was 62 g/L. Laboratory results showed activated partial thromboplastin time (APTT) prolonged with a test value of 83.40 seconds and could not be corrected by corrective experiments; Factor XI activity was less than 0.1%, positive for factor XI antibody, and a plasma coagulation factor XI inhibitor was 35.20 BU/mL. She was eventually diagnosed with acquired coagulation factor XI deficiency, Plasmapheresis were performed to clear antibodies, and patient's condition improved.

Conclusions: Plasmapheresis played a positive role in the treatment of acquired factor XI deficiency. Clear early diagnosis and proper treatment can help to improve the cure rate of patients.

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来源期刊
Clinical laboratory
Clinical laboratory 医学-医学实验技术
CiteScore
1.50
自引率
0.00%
发文量
494
审稿时长
3 months
期刊介绍: Clinical Laboratory is an international fully peer-reviewed journal covering all aspects of laboratory medicine and transfusion medicine. In addition to transfusion medicine topics Clinical Laboratory represents submissions concerning tissue transplantation and hematopoietic, cellular and gene therapies. The journal publishes original articles, review articles, posters, short reports, case studies and letters to the editor dealing with 1) the scientific background, implementation and diagnostic significance of laboratory methods employed in hospitals, blood banks and physicians'' offices and with 2) scientific, administrative and clinical aspects of transfusion medicine and 3) in addition to transfusion medicine topics Clinical Laboratory represents submissions concerning tissue transplantation and hematopoietic, cellular and gene therapies.
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