文章：

种族/民族在系统性AL淀粉样变性中的差异：对疾病及结局不平等的观点

Race/ethnicity in systemic AL amyloidosis: perspectives on disease and outcome disparities

原文发布日期：2020-11-10

DOI: 10.1038/s41408-020-00385-0

类型: Article

开放获取: 是

英文摘要：

In marked contrast to multiple myeloma, racial/ethnic minorities are underrepresented in publications of systemic light-chain (AL) amyloidosis. The impact of race/ethnicity is therefore lacking in the narrative of this disease. To address this gap, we compared disease characteristics, treatments, and outcomes across racial/ethnic groups in a referred cohort of patients with AL amyloidosis from 1990 to 2020. Among 2416 patients, 14% were minorities. Non-Hispanic Blacks (NHBs) comprised 8% and had higher-risk sociodemographic factors. Hispanics comprised 4% and presented with disproportionately more BU stage IIIb cardiac involvement (27% vs. 4–17%). At onset, minority groups were younger in age by 4–6 years. There was indication of more aggressive disease phenotype among NHBs with higher prevalence of difference between involved and uninvolved free light chains >180 mg/L (39% vs. 22–33%, P = 0.044). Receipt of stem cell transplantation was 30% lower in Hispanics compared to non-Hispanic White (NHWs) on account of sociodemographic and physiologic factors. Although the age/sex-adjusted hazard for death among NHBs was 24% higher relative to NHWs (P = 0.020), race/ethnicity itself did not impact survival after controlling for disease severity and treatment variables. These findings highlight the complexities of racial/ethnic disparities in AL amyloidosis. Directed efforts by providers and advocacy groups are needed to expand access to testing and effective treatments within underprivileged communities.
 

摘要翻译： 

与多发性骨髓瘤形成鲜明对比的是，系统性轻链型（AL）淀粉样变性的文献报道中少数种族/族裔群体的代表性不足。因此，关于种族/族裔对该疾病影响的论述颇为缺乏。为填补这一空白，我们比较了1990年至2020年间一个转诊队列中AL淀粉样变性患者不同种族/族裔群体的疾病特征、治疗和结局。在2416例患者中，14%为少数族裔。非西班牙裔黑人占8%，且具有更高风险的社会人口学因素。西班牙裔占4%，其就诊时BU分期IIIb级心脏受累的比例异常高（27% vs. 4-17%）。少数族裔群体发病时年龄更轻，相差4-6岁。有迹象表明非西班牙裔黑人具有更具侵袭性的疾病表型，受累与未受累游离轻链差值>180 mg/L的患病率更高（39% vs. 22-33%, P=0.044）。由于社会人口学和生理因素，西班牙裔患者接受干细胞移植的比例较非西班牙裔白人低30%。尽管经年龄/性别校正后，非西班牙裔黑人的死亡风险相对于非西班牙裔白人高出24%（P=0.020），但在控制疾病严重程度和治疗变量后，种族/族裔本身并不影响生存。这些发现揭示了AL淀粉样变性中种族/族裔差异的复杂性。需要医疗服务提供者和倡导团体采取定向努力，以扩大贫困社区获得检测和有效治疗的机会。

原文链接：

Race/ethnicity in systemic AL amyloidosis: perspectives on disease and outcome disparities