华氏巨球蛋白血症的髓外疾病：一项基于人群的观察性研究。
Extramedullary disease in Waldenström macroglobulinemia: A population-based observational study.
作者信息
Østergaard Simon, Munksgaard Lars, Nielsen Torsten Holm, Hammer Troels, Pedersen Lars Møller, Ølgod Pedersen Mette, Gjerdrum Lise Mette Rahbek
机构信息
Department of Pathology Zealand University Hospital Roskilde Denmark.
Department of Clinical Medicine University of Copenhagen Copenhagen Denmark.
出版信息
EJHaem. 2024 Oct 25;5(6):1269-1273. doi: 10.1002/jha2.1037. eCollection 2024 Dec.
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.
引言
髓外疾病（EMD）是华氏巨球蛋白血症（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患者队列中EMD的临床意义。
结果
30例（6.4%）患者被诊断为EMD，中枢神经系统、肾脏和肺部是最常受累的部位。15年时EMD的累积发病率为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存在持续风险，对长期生存无显著影响。
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