Department of Internal Medicine, Division of Hematology, İstanbul University Cerrahpaşa School of Medicine, İstanbul, Turkey
Department of Internal Medicine, İstanbul University Cerrahpaşa School of Medicine, İstanbul, Turkey
Balkan Med J. 2018 Nov 15;35(6):417-421. doi: 10.4274/balkanmedj.2017.1215. Epub 2018 Jul 3.
Thrombotic thrombocytopenic purpura is a potentially life-threatening condition. Although the introduction of therapeutic plasma exchange has reduced mortality rates from over 90% to 10%-20%, approximately 40% of patients relapse, and outcomes may be fatal in refractory patients. There is clearly a need for additional therapeutic approaches.
To describe the outcomes of relapsed/refractory thrombotic thrombocytopenic purpura patients treated with vincristine as an adjunct to therapeutic plasma exchange.
Cross-sectional study.
The medical records of all relapsed/refractory patients with thrombotic thrombocytopenic purpura treated with vincristine adjunct to therapeutic plasma exchange between October 2000 and December 2016 were retrospectively reviewed. Diagnosis of thrombotic thrombocytopenic purpura was based on clinical history, physical examination, and laboratory examinations. Patient demographics, laboratory findings, initial date and duration of therapeutic plasma exchange, dosage and time of administration of vincristine, and outcomes were recorded.
The study included 15 patients [median age: 37 years (range: 26-65); 7 women and 8 men] with either relapsed or refractory thrombotic thrombocytopenic purpura who were treated with vincristine as an adjunct to therapeutic plasma exchange for a total of 22 episodes. Eighty-seven percent of patients achieved remissions in 20 of 22 episodes, with a median duration of remission of 29.5 months (range: 3-105). After a median follow-up of 55 months, 11 patients were alive. Vincristine was well tolerated with no safety concerns.
Vincristine offers a reasonable option for the treatment of patients with relapsed/refractory thrombotic thrombocytopenic purpura. Further studies evaluating vincristine in the front-line setting and in the relapsed/refractory setting are needed to validate the role of vincristine in thrombotic thrombocytopenic purpura patients.
血栓性血小板减少性紫癜是一种潜在的危及生命的疾病。尽管治疗性血浆置换的引入已将死亡率从超过 90%降至 10%-20%，但仍有约 40%的患者复发，且难治性患者的结局可能是致命的。显然需要额外的治疗方法。
描述用长春新碱作为治疗性血浆置换辅助治疗复发/难治性血栓性血小板减少性紫癜患者的结局。
横断面研究。
回顾性分析 2000 年 10 月至 2016 年 12 月期间用长春新碱辅助治疗性血浆置换治疗的复发/难治性血栓性血小板减少性紫癜患者的所有病历。血栓性血小板减少性紫癜的诊断基于临床病史、体格检查和实验室检查。记录患者的人口统计学、实验室发现、开始治疗性血浆交换的日期和持续时间、长春新碱的剂量和给药时间以及结局。
该研究纳入了 15 例（中位年龄：37 岁（范围：26-65）；7 名女性和 8 名男性）患者，他们均患有复发或难治性血栓性血小板减少性紫癜，共 22 例接受长春新碱辅助治疗性血浆交换。22 例中有 20 例（87%）患者达到缓解，缓解的中位持续时间为 29.5 个月（范围：3-105）。中位随访 55 个月后，11 例患者存活。长春新碱耐受性良好，无安全性问题。
长春新碱为治疗复发/难治性血栓性血小板减少性紫癜患者提供了一种合理的选择。需要进一步研究评估长春新碱在一线治疗和复发/难治性治疗中的作用，以验证其在血栓性血小板减少性紫癜患者中的作用。
