文章：

年轻意义未明单克隆丙种球蛋白病（MGUS）患者的预后

Prognosis of young patients with monoclonal gammopathy of undetermined significance (MGUS)

原文发布日期：2021-02-01

DOI: 10.1038/s41408-021-00406-6

类型: Article

开放获取: 是

英文摘要：

Monoclonal gammopathy of undetermined significance (MGUS) is rare in young patients (age <40 years at diagnosis), with a prevalence of <0.3%, representing ~2% of all patients with MGUS. We hypothesized that MGUS detected in young patients may be associated with a higher risk of progression. We examined 249 patients with MGUS < 40 years old. Among these, 135 patients had immune-related conditions, including infections, autoimmune and inflammatory disorders at the time of diagnosis of MGUS. The risk of progression to multiple myeloma or a related disorder at 5 years and 10 years was 6.0% and 13.8%, respectively. The size of M protein was a significant risk factor for progression (HR 4.2, 95% CI 2.2–7.9). There was a trend that the risk of progression was higher in patients without immune-related conditions (HR 2.36, 95% CI 0.85–6.52, p = 0.088). The M protein resolved in 36 (14%) patients, with a greater likelihood of resolution in patients with immune-related conditions (RR 1.9, 95% CI 1.02–3.6). Young patients with MGUS have a similar risk of progression as older patients, 1.4% per year. Over 50% are diagnosed in the setting of immune-related disorders. Patients with immune-related disorders may have a lower risk of progression.
 

摘要翻译： 

意义未明的单克隆丙种球蛋白病（MGUS）在年轻患者（诊断时年龄<40岁）中较为罕见，患病率低于0.3%，约占所有MGUS患者的2%。我们推测年轻患者中检测到的MGUS可能具有更高的进展风险。我们研究了249例年龄小于40岁的MGUS患者。其中，135例患者在诊断MGUS时患有免疫相关疾病，包括感染、自身免疫性疾病和炎症性疾病。5年和10年内进展为多发性骨髓瘤或相关疾病的风险分别为6.0%和13.8%。M蛋白水平是疾病进展的重要风险因素（风险比4.2，95%置信区间2.2-7.9）。无免疫相关疾病的患者其进展风险有更高趋势（风险比2.36，95%置信区间0.85-6.52，p=0.088）。36例（14%）患者的M蛋白消失，且伴有免疫相关疾病的患者M蛋白消失的可能性更大（相对风险1.9，95%置信区间1.02-3.6）。年轻MGUS患者的年进展风险约为1.4%，与老年患者相似。超过50%的患者是在免疫相关疾病背景下被诊断的。伴有免疫相关疾病的患者可能具有较低的疾病进展风险。

原文链接：

Prognosis of young patients with monoclonal gammopathy of undetermined significance (MGUS)