在患有VEXAS综合征的患者中进行静脉和动脉血栓形成
Yael Kusne1, Atefeh Ghorbanzadeh2, Alina Dulau-Florea3
1Department of Hematology and Oncology, Mayo Clinic, Phoenix, AZ.
Blood
|February 2, 2024
概括
患有VEXAS综合征的患者面临高风险的静脉血栓塞栓症 (VTE),近一半经历血栓形成. 建议对高风险人群进行早期血栓预防,以减轻这些风险.
科学领域:
- 血液学 血液学 血液学
- 类风湿病学 类风湿病学
- 遗传学 是一个遗传学.
背景情况:
- 维克萨斯综合征是UBA1体质突变疾病,呈现出多种系统性症状.
- 血栓形成是VEXAS综合征的一个显著的临床表现.
- 关于血栓形成风险因素和VEXAS综合征发病率的数据有限.
研究的目的:
- 评估VEXAS综合征患者静脉和动脉血栓的频率和结果.
- 为了确定与血栓形成发展相关的临床因素.
- 评估血栓形成对整体存活时间的影响.
主要方法:
- 对119名VEXAS综合征患者的回顾性分析.
- 对静脉血栓栓塞 (VTE) 和动脉血栓发生率的评估.
- 血栓形成与临床表现和生存结果的相关性.
主要成果:
- 血栓形成发生在49%的患者中;VTE发生在41%的患者中,动脉血栓形成发生在6%的患者中.
- 近三分之二的VTE事件是未引起的,20%发生尽管服用抗凝药.
- 心脏和肺炎与静脉瘤相关;M41L突变特别与肺栓塞有关.
结论:
- 维克萨斯综合征患者的VTE累积发病率很高,需要考虑血栓预防.
- 在患有血栓形成和没有血栓形成的患者之间,没有观察到总生存时间的显著差异.
- 需要进一步研究VTE风险分层和VEXAS综合征的管理.
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