Extramedullary disease in Waldenström macroglobulinemia: A population-based observational study

EJHaem Pub Date : 2024-10-25 DOI:10.1002/jha2.1037
Simon Østergaard, Lars Munksgaard, Torsten Holm Nielsen, Troels Hammer, Lars Møller Pedersen, Mette Ølgod Pedersen, Lise Mette Rahbek Gjerdrum
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Abstract

Introduction: Extramedullary disease (EMD) is a rare manifestation of Waldenström macroglobulinemia (WM), and its clinical and prognostic implications are poorly understood. Methods: In this single-center study, we investigated the clinical significance of EMD in a cohort of 469 WM patients. Results: EMD was identified in 30 (6.4%) patients, with the central nervous system, kidneys, and lungs being the most frequently affected sites. The cumulative incidence of EMD was 12.6% at 15 years. Median overall survival rates at 5 and 10 years for patients with EMD were 63% and 37%, respectively. Conclusion: Our findings indicate a persistent risk of EMD throughout the disease course, with no significant impact on long-term survival.

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Waldenström巨球蛋白血症的髓外疾病:一项基于人群的观察性研究
髓外疾病(EMD)是Waldenström巨球蛋白血症(WM)的一种罕见表现,其临床和预后意义尚不清楚。方法:在这项单中心研究中,我们调查了469例WM患者中EMD的临床意义。结果:30例(6.4%)患者发现EMD,其中中枢神经系统、肾脏和肺部是最常见的受累部位。15年时EMD的累计发病率为12.6%。EMD患者5年和10年的中位总生存率分别为63%和37%。结论:我们的研究结果表明,在整个疾病过程中,EMD的风险持续存在,对长期生存没有显著影响。
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