粉样粉症和多发性骨髓瘤:一个复杂的情景,其中心脏参与仍然是关键的预后因素
Rafael Ríos-Tamayo1, Isabel Krsnik1, Manuel Gómez-Bueno1
1Hospital Universitario Puerta de Hierro, IDIPHISA, CIBERCV, 28222 Majadahonda, Spain.
Life (Basel, Switzerland)
|July 29, 2023
概括
系统性轻链氨基粉症 (AL) 和多发性骨髓瘤 (MM) 的同时发生在141名患者中进行了研究. AL/MM关联似乎是同步的,并影响了生存率,尽管不是显著的,这突显了需要进一步研究的需要.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 心脏病学 心脏病学
背景情况:
- 单克隆性淋巴病变 (MGs) 可以演变,并发性MGs等并发性疾病是显著的.
- 系统性轻链 (AL) 氨基粉症和多发性骨髓瘤 (MM) 之间的联系缺乏广泛的证据.
研究的目的:
- 调查同时发生的AL氨基粉症和多发性骨髓瘤 (AL/MM) 的特征和预后影响.
主要方法:
- 从2005年1月到2023年4月的141名AL氨基粉症患者的预期招生.
- 在AL氨基粉症患者中对多发性髓瘤 (MM) 的诊断标准的评估.
- 生存分析比较AL/MM患者与仅有AL的患者.
主要成果:
- 18.7%的AL氨基粉症患者符合MM (AL/MM) 的标准.
- 同步的AL / MM比连续发生更频繁.
- 虽然AL/MM患者的整体存活率有所下降,但这种差异在统计学上并不显著.
- 年龄,NT-proBNP水平和自身干细胞移植是AL Amyloidosis的独立预后因素.
结论:
- 观察到AL/MM关联在AL氨基粉症患者的显著亚组中.
- 虽然在这个系列中没有统计学意义,但由于潜在的生存影响,AL / MM关联需要进一步调查.
- 无论MM是否同时发生,心脏参与仍然是AL Amyloidosis的主要预后因素.
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