A型血友病致难治性鼻出血1例并文献复习

doi:10.6040/j.issn.1673-3770.0.2023.251

摘要/Abstract

摘要： 目的探讨1例临床较少见的A型血友病难治性鼻出血的临床特征、实验室检查及诊疗过程。方法通过回顾分析患者的病例资料,总结病例特点及治疗过程中的经验与不足,并总结国内外血友病难治性鼻出血相关文献。结果患者首发症状为自发性鼻出血,无鼻腔、鼻咽部占位病变,起初于门诊行鼻内镜下电凝止血治疗,症状先缓解后立即恶化,入院后经历前后鼻孔填塞、输注凝血因子后仍未缓解,遂进一步行介入治疗后,得以控制症状。术后仅数小时,再次出血,遂于全麻下将下鼻道扩容后行“鼻腔电凝术+前鼻孔填塞术”,方才成功止血,随访至今未再次出血。结论血友病是一类具有 X染色体隐性遗传特征的先天性出血性疾病,鼻出血是其主要症状之一。临床上发现无明显诱因鼻出血,经治疗后反复发作不愈,均应考虑到血友病的可能。血友病鼻出血发展为难治性鼻出血后,由于凝血因子大量消耗,需及时补充凝血因子,该类患者在行介入治疗时,由于术前可能已行鼻腔填塞或其他治疗,介入手术虽止血成功率较高,但术后止血后,仍需再次行鼻内镜检查,将可疑出血位置烧灼止血,避免术后二次出血。同时在专科治疗无效时应尽快借助多学科协作诊疗确认出血原因,提高临床疗效。

关键词: 血友病A型, 难治性鼻出血, 鼻内镜, 血管造影, 输血

Abstract: Objective This study aims to examine the clinical features and methods of laboratory examination for the diagnosis of a rare hemophilia refractory epistaxis. Methods We reviewed and analyzed the patient's case data, summarized the characteristics of the case, and noted the deficiencies in the treatment process. Local and international literatures on hemophiliac rhinorrhea were also reviewed in the domestic and overseas. Results The patient presented with epistaxis without apparent cause as the first symptom, and have no space occupying lesions of the nasal cavity and nasopharynx. Initially, the patient underwent electrocoagulation treatment in the outpatient department, but the symptoms worsened immediately. After admission, the symptoms were still not relieved after undergoing anterior and posterior nasal tamponade and transfusion of coagulation factors. Only a few hours after the operation, the condition worsened again. After nasal plasma electrocoagulation under general anesthesia, the bleeding was successfully stopped and has not recurred thus far. Conclusion Hemophilia is a type of congenital hemorrhagic disease with X-linked recessive inheritance, and a nosebleed is one of its main symptoms. The possibility of hemophilia should be taken into account in patients who have nosebleeds that do not have an obvious cause and persist after treatment. After hemophilia develops into refractory nosebleed, it is necessary to supplement the coagulation factors in time due before they are depleted. During interventional therapy, such patients may have received nasal tamponade or other treatments before surgery. Although the success rate of hemostasis after interventional surgery is relatively high, nasal endoscopy should be performed again after postoperative hemostasis. The suspected bleeding site is cauterized to stop the bleeding and avoid postoperative secondary bleeding. At the same time, a multidisciplinary team must be formed to identify the cause of bleeding and improve the clinical efficacy when the specialized treatment fails.

Key words: Hemophilia A, Refractory epistaxis, Nasal endoscopy, Angiography, Blood transfusion

中图分类号:

R765.23

郑露,程杰,欧阳虹,池花明,杜敬东. A型血友病致难治性鼻出血1例并文献复习[J]. 山东大学耳鼻喉眼学报, 2024, 38(5): 86-90.

ZHENG Lu, CHENG Jie, OUYANG Hong, CHI Huaming, DU Jingdong. Intractable nosebleed due to haemophilia A: a case report and literature review[J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2024, 38(5): 86-90.

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