双倍意味着麻烦吗? 骨髓增殖和淋巴增殖新生体的共存
Danijela Lekovic1,2, Jelena Ivanovic1, Tatjana Terzic3
1Clinic of Hematology, University Clinical Center of Serbia, 11000 Belgrade, Serbia.
Journal of clinical medicine
|March 28, 2024
概括
骨髓增殖性瘤 (MPNs) 和淋巴增殖性瘤 (LPNs) 的罕见同时发生,具有侵略性的临床过程和增加的血栓形成风险. 在MPN患者中早期发现LPN对于改善结果至关重要.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 新生病性疾病 新生病性疾病
背景情况:
- 已知骨髓增殖性瘤 (MPNs) 和淋巴增殖性瘤 (LPNs) 独立进化.
- 许多PNs和LPNs的共存是罕见的,对它们的综合特征和长期结果的理解有限.
研究的目的:
- 分析MPN和LPN同时存在的患者的临床特征和长期随访.
主要方法:
- 在过去二十年中,在一个大学中心对14名被诊断为MPN/LPN共存的患者进行了回顾性分析.
- 根据既定的临床指南诊断和治疗患者.
主要成果:
- 患者的平均年龄为53岁. 患有LPN的MPN患者经历了较短的潜伏期到第二次恶性瘤和更积极的LPN过程.
- 多细胞血症和慢性淋巴细胞白血病是最常见的相关疾病 (36%). 在2/3的患者中存在JAK2V617F突变,在1/3的患者中存在细胞遗传异常.
- MPN/LPN共存与显著更高的血栓形成风险 (42.8%) 和更高的第三恶性瘤发育频率 (21.4%) 相关.
结论:
- MPN/LPN的共存往往导致侵袭性疾病和增加的血栓形成风险.
- 建议在MPN患者中早期检测LPN,监测淋巴细胞病,淋巴腺病和快速有机巨变.
- 分子和细胞遗传异常表明遗传不稳定性,增加多种瘤和血栓事件的风险.
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