继发于胆管癌的获得性血友病A 1例报告并文献复习。

Q3 Medicine
Xiaoting Han, Lei Fu, Liang Li, Jianjun Bian, Mei Zhao, Guobin Bi
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引用次数: 0

摘要

获得性血友病A (AHA)是一种罕见的自身免疫性出血性疾病。继发于肝胆恶性肿瘤更是罕见,若不及时诊断和治疗,死亡率极高。有必要提高对这种疾病的认识。本文报告一例70岁女性患者,于2022年10月在蚌埠医学院第二附属医院就诊,胆管癌术后2个月确诊为AHA。患者表现为双下肢皮下血肿。凝血功能试验显示活化部分凝血活素时间(APTT)明显延长74.5秒,APTT混合试验无修正。凝血因子测定显示凝血因子VIII活性(FVIII:C)严重降低0.3%,抑制剂滴度为25.6 BU/mL。在排除其他潜在原因后,患者被诊断为胆管癌相关AHA。通过化疗控制原发肿瘤,加上止血和免疫抑制治疗消除抑制剂,AHA得到控制。患者无进一步凝血异常或出血,可及时、全程化疗胆管癌,显著提高生存率和生活质量。因此,对于在手术、创伤或侵入性手术后出现自发性出血或异常出血的恶性肿瘤患者,临床医生应警惕继发性AHA的可能性。及时诊断和治疗可显著改善预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Acquired hemophilia A secondary to cholangiocarcinoma: A case report and literature review.

Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder. Its occurrence secondary to hepatobiliary malignancies is even rarer, and without timely diagnosis and treatment, the mortality rate is extremely high. There is a need to raise awareness of this disease. This report describes a case of a 70-year-old female patient diagnosed with AHA 2 months after surgery for cholangiocarcinoma, admitted to the Second Affiliated Hospital of Bengbu Medical College in October 2022. The patient presented with subcutaneous hematoma in both lower limbs. Coagulation function tests showed a markedly prolonged activated partial thromboplastin time (APTT) of 74.5 seconds, with no correction in the APTT mixing test. Coagulation factor assays revealed a severely reduced coagulation factor VIII activity (FVIII:C) of 0.3%, and an inhibitor titer of 25.6 BU/mL was detected. After ruling out other potential causes, the patient was diagnosed with cholangiocarcinoma-associated AHA. With chemotherapy to control the primary tumor, alongside hemostatic and immunosuppressive therapy for inhibitor eradication, AHA was brought under control. The patient had no further coagulation abnormalities or bleeding, enabling timely and full-course chemotherapy for cholangiocarcinoma and significantly improving survival and quality of life. Therefore, in patients with malignancies who present with spontaneous bleeding or unusual bleeding following surgery, trauma, or invasive procedures, clinicians should be alert to the possibility of secondary AHA. Timely diagnosis and treatment can significantly improve prognosis.

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来源期刊
中南大学学报(医学版)
中南大学学报(医学版) Medicine-Medicine (all)
CiteScore
1.00
自引率
0.00%
发文量
8237
期刊介绍: Journal of Central South University (Medical Sciences), founded in 1958, is a comprehensive academic journal of medicine and health sponsored by the Ministry of Education and Central South University. The journal has been included in many important databases and authoritative abstract journals at home and abroad, such as the American Medline, Pubmed and its Index Medicus (IM), the Netherlands Medical Abstracts (EM), the American Chemical Abstracts (CA), the WHO Western Pacific Region Medical Index (WPRIM), and the Chinese Science Citation Database (Core Database) (CSCD); it is a statistical source journal of Chinese scientific and technological papers, a Chinese core journal, and a "double-effect" journal of the Chinese Journal Matrix; it is the "2nd, 3rd, and 4th China University Excellent Science and Technology Journal", "2008 China Excellent Science and Technology Journal", "RCCSE China Authoritative Academic Journal (A+)" and Hunan Province's "Top Ten Science and Technology Journals". The purpose of the journal is to reflect the new achievements, new technologies, and new experiences in medical research, medical treatment, and teaching, report new medical trends at home and abroad, promote academic exchanges, improve academic standards, and promote scientific and technological progress.
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