Division of Hematology, Massachusetts General Hospital, Harvard Medical School, Boston, MA.
Division of Pulmonary and Respiratory Diseases, Boston Children's Hospital, Harvard Medical School, Boston, MA; Division of Pulmonary and Critical Care Medicine, Brigham & Women's Hospital, Harvard Medical School, Boston, MA.
Chest. 2019 Jun;155(6):1226-1233. doi: 10.1016/j.chest.2019.02.010. Epub 2019 Feb 18.
Hemoptysis is a major cause of morbidity and mortality in patients with cystic fibrosis (CF). Antifibrinolytic agents have shown efficacy in a broad range of bleeding disorders and conditions.
The goal of this study was to examine the use of antifibrinolytic agents in managing hemoptysis in CF. We developed a clinical treatment pathway for inpatient and outpatient use, and rates of admission for bleeding prior to and following implementation of the pathway are reported.
All adult patients with CF treated with systemic antifibrinolytic agents over a 54-month period according to the treatment pathway were analyzed. Data collected included demographic characteristics, baseline CF-related characteristics, and bleeding and treatment parameters. Effectiveness of the pathway was evaluated via comparison of annualized hemoptysis admission rates prior to and following pathway enrollment.
Seventy-two distinct episodes of hemoptysis treated with antifibrinolytic agents were analyzed in a total of 21 adult patients with CF. Two-thirds of episodes treated involved moderate or massive hemoptysis. Bleeding ceased following a median of 2 days. Outpatient treatment was associated with a 50% reduction in the annualized hemoptysis admission rate following pathway enrollment (2.44 vs 1.23 admissions per year; P = .0024) that was independent of other changes in management. Antifibrinolytic therapy was well tolerated. One central catheter-associated upper extremity DVT was observed in a patient with previous thrombosis in the same vessel.
A pathway using systemic antifibrinolytic therapy to treat hemoptysis in patients with CF was associated with a reduction in hospital admissions. No serious adverse events were observed. Additional studies are needed to further define the benefits of systemic antifibrinolytic use in patients with CF.
咯血是囊性纤维化（CF）患者发病率和死亡率的主要原因。抗纤维蛋白溶解剂已在广泛的出血性疾病和病症中显示出疗效。
本研究旨在研究抗纤维蛋白溶解剂在 CF 咯血管理中的应用。我们制定了住院和门诊使用的临床治疗途径，并报告了在实施该途径之前和之后因出血而入院的比率。
根据治疗途径，分析了在 54 个月期间接受全身性抗纤维蛋白溶解剂治疗的所有成年 CF 患者。收集的数据包括人口统计学特征、基线 CF 相关特征以及出血和治疗参数。通过比较途径注册前后的年化咯血入院率来评估途径的有效性。
在总共 21 名 CF 成年患者中，分析了 72 个用抗纤维蛋白溶解剂治疗的不同咯血发作。三分之二的治疗发作涉及中度或大量咯血。出血中位数在 2 天后停止。门诊治疗与途径注册后年化咯血入院率降低 50%相关（2.44 次/年与 1.23 次/年；P=.0024），这与其他治疗方法的改变无关。抗纤维蛋白溶解疗法耐受性良好。在同一血管中有先前血栓形成的患者中观察到一个中心导管相关的上肢 DVT。
使用全身性抗纤维蛋白溶解疗法治疗 CF 患者咯血的途径与住院人数减少相关。未观察到严重不良事件。需要进一步的研究来进一步确定 CF 患者全身性抗纤维蛋白溶解剂使用的益处。
