Race/ethnicity in systemic AL amyloidosis: perspectives on disease and outcome disparities.
Race/ethnicity in systemic AL amyloidosis: perspectives on disease and outcome disparities.
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全身性淀粉样变性的种族/种族：疾病和结果差异的观点。
DOI：
10.1038/s41408-020-00385-0
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发表时间：
2020-11-10
影响因子：
12.8
通讯作者：
Sanchorawala V
中科院分区：
文献类型：
--
作者：
Staron A;Connors LH;Zheng L;Doros G;Sanchorawala V
与多发性骨髓瘤形成鲜明对比的是，少数族裔在系统性轻链 (AL) 淀粉样变性的出版物中代表性不足。因此，在这种疾病的叙述中缺乏种族/民族的影响。为了解决这一差距，我们比较了 1990 年至 2020 年 AL 淀粉样变性患者转介队列中不同种族/民族的疾病特征、治疗和结果。在 2416 名患者中，14% 是少数族裔。非西班牙裔黑人 (NHB) 占 8%，具有较高风险的社会人口因素。西班牙裔占 4%，且 BU IIIb 期心脏受累比例更高（27% vs. 4-17%）。少数群体发病时的年龄要小 4-6 岁。有迹象表明，NHB 中的疾病表型更具侵袭性，涉及和未涉及的游离轻链之间的差异 >180mg/L 的患病率更高（39% vs. 22–33%，P=0.044）。由于社会人口统计和生理因素，西班牙裔干细胞移植的接受率比非西班牙裔白人 (NHW) 低 30%。尽管 NHB 的年龄/性别调整死亡风险相对于 NHW 高 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。这些发现凸显了 AL 淀粉样变性中种族/民族差异的复杂性。医疗服务提供者和倡导团体需要作出直接努力，以扩大贫困社区获得检测和有效治疗的机会。
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.
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