在慢性粒状瘤疾病中连续的非Aspergillus真菌侵入性感染:来自法国国家初级免疫缺陷参考中心的数据和文献综述
Leïla Lefevre1,2, Olivier Paccoud1,2, Bénédicte Neven1,3,4
1Paris Cité University, Paris, France.
Journal of clinical immunology
|November 5, 2025
概括
慢性花瘤疾病 (CGD) 患者的非阿斯伯吉勒斯侵入性真菌感染 (NAFI) 是严重的,通常需要侵入性诊断. 造血干细胞移植 (HSCT) 为这些具有挑战性的真菌感染提供了有效的管理.
科学领域:
- 免疫学 免疫学 免疫学
- 传染性疾病 传染性疾病
- 血液学 血液学 血液学
背景情况:
- 非Aspergillus侵入性真菌感染 (NAFI) 越来越多地在患有慢性花瘤病 (CGD) 的患者中被识别出来.
- 对CGD患者的NAFI的精确临床描述和管理策略仍然有限.
研究的目的:
- 通过使用法国国家原发性免疫缺陷注册表 (CEREDIH) 对CGD患者的NAFI病例进行回顾性分析.
- 进行全面的文献审查,以进一步描述CGD.中的NAFI.
主要方法:
- 来自CEREDIH注册表的16个经过验证的NAFI案件的回顾性分析.
- 从文献综述中包括106个可能/已证实的NAFI案例.
- 分析感染类型 (菌,酵母,肺囊),患者人口统计,治疗,诊断方法和结果.
主要成果:
- 总共分析了122个NAFI病例,主要是菌 (84例) 和酵母 (35例).
- 模具NAFI发生在平均年龄为17岁,经常影响肺部,经常呈现为突破性感染.
- 酵母菌感染发生较早 (平均年龄为5岁),经常传播,菌和酵母菌NAFI的死亡率约为25%,尽管包括HSCT在内的治疗方法.
结论:
- 在CGD患者中,NAFI是严重的,尽管预防和额外的免疫抑制,但经常发生.
- 诊断通常需要侵入性手术,血造干细胞移植 (HSCT) 显示出治疗这些感染的前景.
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