Ríos-Tamayo Rafael, Krsnik Isabel, Gómez-Bueno Manuel, Garcia-Pavia Pablo, Segovia-Cubero Javier, Huerta Ana, Salas Clara, Silvestre Ramona Ángeles, Sánchez Amelia, Manso Marta, Delgado Laura, Lahuerta Juan José, Martínez-López Joaquín, Duarte Rafael F
Hospital Universitario Puerta de Hierro, IDIPHISA, CIBERCV, 28222 Majadahonda, Spain.
Hospital Universitario 12 de Octubre, Instituto de Investigación del Hospital Universitario 12 de Octubre, 28041 Madrid, Spain.
Life (Basel). 2023 Jul 6;13(7):1518. doi: 10.3390/life13071518.
Monoclonal gammopathies (MGs) are a wide range of diseases that may evolve or progress over time. Comorbidity plays a critical role in this setting. The co-occurrence of two MGs is not a rare event. The evidence on the association of systemic light chain (AL) amyloidosis and multiple myeloma (MM) is scarce and controversial. Herein we aim to address this topic in a large series of patients of a referral center. All consecutive AL amyloidosis patients treated at our center from January 2005 to April 2023 were prospectively enrolled in a clinical and epidemiological registry. 141 patients diagnosed with AL amyloidosis were included, of which 7 (5%) had localized whereas 134 presented with systemic disease. The heart was the most frequently affected organ (90.3%). 25 patients (18.7%) fulfilled the IMWG diagnostic criteria of MM (AL/MM). Time-dependent association between AL and MM showed that the synchronous pattern is more frequent than the appearance of a second primary malignancy. The diagnostic delay was six months (m). Patients with AL/MM had a poorer median overall survival (OS) than AL-only patients (35.5 m, CI 95% 0-88.9, vs. 52.6 m, CI 95% 16.7-88.5), but this difference was not statistically significant. The prognosis in AL is dominated by the heart involvement, which is massive in this series. In our Cox regression model, only three prognostic variables remain as independent prognostic factors: age, N-terminal pro-brain natriuretic peptide (≥8500 ng/L), and undergoing an autologous stem cell transplant, whereas left ventricular ejection fraction shows a marginal effect. More and large studies focusing on the AL/MM association are needed to uncover the characteristics and prognostic impact of this association.
单克隆丙种球蛋白病(MGs)是一大类可能随时间演变或进展的疾病。合并症在这种情况下起着关键作用。两种MGs同时出现并非罕见事件。关于系统性轻链(AL)淀粉样变性与多发性骨髓瘤(MM)关联的证据稀少且存在争议。在此,我们旨在对一家转诊中心的大量患者进行该主题的研究。2005年1月至2023年4月在我们中心接受治疗的所有连续AL淀粉样变性患者均被前瞻性纳入临床和流行病学登记。纳入了141例诊断为AL淀粉样变性的患者,其中7例(5%)为局限性病变,134例为系统性疾病。心脏是最常受累的器官(90.3%)。25例患者(18.7%)符合MM(AL/MM)的国际骨髓瘤工作组(IMWG)诊断标准。AL与MM的时间依赖性关联表明,同步模式比第二种原发性恶性肿瘤的出现更为常见。诊断延迟为6个月。AL/MM患者的中位总生存期(OS)比单纯AL患者差(35.5个月,95%置信区间0 - 88.9,对比52.6个月,95%置信区间16.7 - 88.5),但这种差异无统计学意义。AL的预后主要受心脏受累影响,在本系列中这种情况很严重。在我们的Cox回归模型中,仅三个预后变量作为独立预后因素保留下来:年龄、N末端脑钠肽前体(≥8500 ng/L)以及接受自体干细胞移植,而左心室射血分数显示出边际效应。需要更多大规模研究关注AL/MM关联,以揭示这种关联的特征和预后影响。