Thrombosis and Hemostasis Research Unit, Department of Clinical Biochemistry, Aarhus University Hospital, Aarhus, Denmark.
Department of Clinical Biochemistry, Viborg Regional Hospital, Viborg, Denmark.
Semin Thromb Hemost. 2021 Jul;47(5):581-588. doi: 10.1055/s-0040-1715795. Epub 2020 Sep 23.
Solid malignant neoplasms have the capability of disturbing the fibrinolytic system, leading to primary hyperfibrinolysis, a paraneoplastic syndrome that potentially results in severe bleeding. Yet, the full extent of primary hyperfibrinolysis in solid malignant neoplasms is unknown. Thus, the purpose of this study was to systematically review the current literature regarding clinical manifestations, biochemical diagnosis, and treatment of primary hyperfibrinolysis in patients with solid malignant neoplasms. The review was performed in agreement with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The databases PubMed, Embase, Scopus, and Web of Science were searched on December 5, 2019, without time limits. Studies were included if they comprised at least one biochemical marker of fibrinolysis in addition to fibrinogen degradation products such as D-dimer, and furthermore included a correlation between biochemical marker and clinical outcome. In total, 12 studies were included. All studies were case reports including a total of 21 patients. Prostate cancer was the most frequently represented cancer type (76%), and the majority of cancer patients had metastatic disease (81%). Spontaneous bleeding was the clinical presentation in the majority of patients (76%), and the most frequently localization for the bleedings was subcutaneous. Antifibrinolytic agents were the most commonly used treatment and ceased bleedings in 80% of patients. Three patients died of uncontrolled bleedings. In conclusion, primary hyperfibrinolysis induced by solid malignant neoplasms is a rare but potentially life-threatening condition that should be considered, especially in patients with metastatic disease presenting with serious, spontaneous subcutaneous bleedings. A standardized diagnostic strategy is strongly needed.
实体恶性肿瘤具有扰乱纤维蛋白溶解系统的能力，导致原发性纤维蛋白溶解亢进，这是一种潜在的副肿瘤综合征，可能导致严重出血。然而，实体恶性肿瘤中原发性纤维蛋白溶解亢进的全部程度尚不清楚。因此，本研究的目的是系统地回顾目前关于实体恶性肿瘤患者原发性纤维蛋白溶解亢进的临床表现、生化诊断和治疗的文献。该综述是按照系统评价和荟萃分析的首选报告项目（PRISMA）指南进行的。于 2019 年 12 月 5 日在 PubMed、Embase、Scopus 和 Web of Science 数据库中进行了检索，没有时间限制。如果研究除纤维蛋白原降解产物（如 D-二聚体）外还包含至少一种纤维蛋白溶解的生化标志物，并且进一步包含生化标志物与临床结果之间的相关性，则将其纳入研究。共纳入 12 项研究。所有研究均为病例报告，共纳入 21 例患者。前列腺癌是最常见的代表性癌症类型（76%），大多数癌症患者患有转移性疾病（81%）。自发性出血是大多数患者的临床表现（76%），出血最常见的部位是皮下。抗纤维蛋白溶解剂是最常用的治疗方法，80%的患者停止了出血。3 例患者死于无法控制的出血。总之，实体恶性肿瘤引起的原发性纤维蛋白溶解亢进是一种罕见但潜在危及生命的疾病，应予以考虑，尤其是在患有转移性疾病且出现严重自发性皮下出血的患者中。强烈需要制定标准化的诊断策略。
