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因子 XIII 活性降低和触珠蛋白可能是增强型纤溶型弥散性血管内凝血状态的标志物:病例报告。

Decreased factor XIII activity and haptoglobin may be markers of enhanced-fibrinolytic-type disseminated intravascular coagulation status: Case report.

作者信息

Yamada Shinya, Ueda Hideyasu, Suga Yukio, Miyamoto Toshihiro, Asakura Hidesaku

机构信息

Department of Hematology, Kanazawa University Hospital, Kanazawa, Japan.

Department of Cardiovascular Surgery, Kanazawa University Hospital, Kanazawa, Japan.

出版信息

Medicine (Baltimore). 2025 Jul 25;104(30):e43123. doi: 10.1097/MD.0000000000043123.

DOI:10.1097/MD.0000000000043123
PMID:40725922
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12303462/
Abstract

RATIONALE

Aortic aneurysms are often accompanied by enhanced-fibrinolytic-type disseminated intravascular coagulation (DIC), but markers to adequately assess DIC status remain lacking.

PATIENT CONCERNS

An 80-year-old man scheduled for thoracic endovascular aortic repair for aortic aneurysm was diagnosed with enhanced-fibrinolytic-type DIC. The concern was whether surgery could be safely performed in a state of enhanced-fibrinolytic-type DIC with a bleeding tendency.

INTERVENTIONS

He was treated with a combination of heparin and tranexamic acid.

OUTCOMES

DIC was well controlled and the surgery was performed safely. However, DIC worsened again postoperatively. Since no severe bleeding symptoms were observed, no therapeutic intervention for DIC was performed postoperatively. During this period, coagulation tests including factor XIII activity and haptoglobin were followed over time to clarify the relevance of these markers.Factor XIII activity was decreased and haptoglobin was mildly decreased in DIC. Improvement of DIC led to normalization of both markers. Re-exacerbation of DIC led to decreased factor XIII activity and mildly decreased haptoglobin.

LESSONS

Factor XIII activity and haptoglobin appear to represent useful markers of DIC status.

摘要

原理

主动脉瘤常伴有纤维蛋白溶解增强型弥散性血管内凝血(DIC),但仍缺乏能充分评估DIC状态的标志物。

患者情况

一名80岁男性因主动脉瘤计划接受胸段血管腔内主动脉修复术,被诊断为纤维蛋白溶解增强型DIC。担心的是在有出血倾向的纤维蛋白溶解增强型DIC状态下能否安全进行手术。

干预措施

对他采用肝素和氨甲环酸联合治疗。

结果

DIC得到良好控制,手术安全进行。然而,术后DIC再次恶化。由于未观察到严重出血症状,术后未对DIC进行治疗干预。在此期间,随时间跟踪包括因子 XIII 活性和触珠蛋白在内的凝血试验,以阐明这些标志物的相关性。在DIC中,因子 XIII 活性降低,触珠蛋白轻度降低。DIC的改善导致这两种标志物均恢复正常。DIC再次加重导致因子 XIII 活性降低,触珠蛋白轻度降低。

经验教训

因子 XIII 活性和触珠蛋白似乎是DIC状态的有用标志物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8ed/12303462/c3b5461d5f7d/medi-104-e43123-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8ed/12303462/c3b5461d5f7d/medi-104-e43123-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8ed/12303462/c3b5461d5f7d/medi-104-e43123-g001.jpg

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本文引用的文献

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Clinical practice guidelines for management of disseminated intravascular coagulation in Japan 2024. Part 3: solid cancers and vascular abnormalities.《2024年日本弥散性血管内凝血管理临床实践指南》。第3部分:实体癌与血管异常
Int J Hematol. 2025 May;121(5):622-632. doi: 10.1007/s12185-024-03912-y. Epub 2025 Jan 10.
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How We Interpret Thrombosis with Thrombocytopenia Syndrome?我们如何解读血栓性血小板减少性紫癜?
Int J Mol Sci. 2024 May 1;25(9):4956. doi: 10.3390/ijms25094956.
3
Distinguishing immune-mediated thrombotic thrombocytopenic purpura from septic disseminated intravascular coagulation using plasma levels of haptoglobin and factor XIII activity.
利用血浆中触珠蛋白水平和因子 XIII 活性区分免疫介导的血栓性血小板减少性紫癜与脓毒症弥散性血管内凝血
Res Pract Thromb Haemost. 2023 Feb 8;7(2):100076. doi: 10.1016/j.rpth.2023.100076. eCollection 2023 Feb.
4
Higher serum haptoglobin levels were associated with improved outcomes of patients with septic shock.血清触珠蛋白水平较高与感染性休克患者的预后改善相关。
Crit Care. 2022 May 17;26(1):131. doi: 10.1186/s13054-022-04007-y.
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Therapeutic Strategies for Disseminated Intravascular Coagulation Associated with Aortic Aneurysm.主动脉瘤相关弥散性血管内凝血的治疗策略。
Int J Mol Sci. 2022 Jan 24;23(3):1296. doi: 10.3390/ijms23031296.
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Mortality at 180-days is affected by serum haptoglobin levels in septic patients with high magnitude serum high mobility group box-1 levels.血清高迁移率族蛋白B1水平较高的脓毒症患者中,180天死亡率受血清触珠蛋白水平影响。
Acute Med Surg. 2022 Feb 1;9(1):e726. doi: 10.1002/ams2.726. eCollection 2022 Jan-Dec.
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Management of disseminated intravascular coagulation associated with aortic aneurysm and vascular malformations.主动脉瘤和血管畸形相关弥散性血管内凝血的处理。
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Mol Aspects Med. 2020 Jun;73:100851. doi: 10.1016/j.mam.2020.100851. Epub 2020 Jul 11.
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