横纹肌肉瘤治疗期间的纤溶亢进。
Hyperfibrinolysis during the treatment of rhabdomyosarcoma.
发表日期:2024 May
作者:
Samuel R Taylor, Jean M Connors, Vinayak Venkataraman
来源:
Research and Practice in Thrombosis and Haemostasis
摘要:
肺泡型横纹肌肉瘤 (ARMS) 中经常观察到凝血病,其中最常见的表现是弥散性血管内凝血 (DIC)。然而,纤溶亢进代表了凝血障碍的一个独特但经常重叠且可能危及生命的子集,需要特定的诊断和治疗方法。临床医生如何识别纤溶亢进以及对治疗的影响是什么?本病例报告描述了一名 25 岁男性患有前列腺转移性 ARMS,在开始化疗一周后出现持续性肉眼血尿。进行全面的凝血检查,包括血小板计数、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的评估。治疗包括补充纤维蛋白原和使用抗纤溶药物。认识到 ARMS 患者的纤溶亢进对于适当的治疗至关重要。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,临床医生应对纤溶亢进保持高度怀疑。在原发性纤溶亢进的情况下,可以考虑使用抗纤溶药物,但它们通常在 DIC 中是禁忌的。© 2024 作者。
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.How can clinicians identify hyperfibrinolysis and what are the implications for management?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.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.© 2024 The Author(s).