治疗横纹肌肉瘤期间的高纤维蛋白溶解症

IF 3.4 3区 医学 Q2 HEMATOLOGY Research and Practice in Thrombosis and Haemostasis Pub Date : 2024-05-01 DOI:10.1016/j.rpth.2024.102467
Samuel R. Taylor , Jean M. Connors , Vinayak Venkataraman
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引用次数: 0

摘要

背景肺泡横纹肌肉瘤(ARMS)中经常出现凝血功能障碍,其中弥散性血管内凝血(DIC)是最常见的表现。关键临床问题临床医生如何识别高纤维蛋白溶解症,其对治疗的影响如何?患者接受了全面的凝血检查,包括评估血小板计数、凝血酶原时间、活化部分凝血活酶时间、纤维蛋白原、D-二聚体和纤维蛋白降解产物。处理方法包括补充纤维蛋白原和使用抗纤维蛋白溶解剂。对于出现严重凝血异常的 ARMS 患者,尤其是前列腺受累或正在接受化疗的患者,临床医生应高度怀疑纤溶亢进。在原发性纤溶亢进的病例中,可考虑使用抗纤溶药物,但在 DIC 中一般禁用抗纤溶药物。
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Hyperfibrinolysis during the treatment of rhabdomyosarcoma

Background

Coagulopathies are frequently observed in alveolar rhabdomyosarcoma (ARMS), with disseminated intravascular coagulation (DIC) being the most common presentation. However, hyperfibrinolysis represents a distinct but often overlapping and potentially life-threatening subset of coagulation disorders that requires specific diagnostic and management approaches.

Key Clinical Question

How can clinicians identify hyperfibrinolysis and what are the implications for management?

Clinical Approach

This case report describes a 25-year-old man with metastatic ARMS arising from the prostate who developed persistent gross hematuria one week after initiating chemotherapy. A comprehensive coagulation workup was performed, including assessment of platelet count, prothrombin time, activated partial thromboplastin time, fibrinogen, D-dimer, and fibrin degradation products. Management included repletion of fibrinogen and the use of anti-fibrinolytic agents.

Conclusion

Recognizing hyperfibrinolysis in ARMS patients is crucial for appropriate management. Clinicians should maintain a high index of suspicion for hyperfibrinolysis in ARMS patients presenting with severe coagulation abnormalities, particularly those with prostatic involvement or undergoing chemotherapy. In cases of primary hyperfibrinolysis, antifibrinolytic agents may be considered, whereas they are generally contraindicated in DIC.

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来源期刊
CiteScore
5.60
自引率
13.00%
发文量
212
审稿时长
7 weeks
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