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

Abstract

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.

Light Chain Amyloidosis: Epidemiology, Staging, and Prognostication

“…Systematic underdetection is also thought to be a large part of the problem because the structural heart changes, proteinuria, and neuropathy seen with AL amyloidosis can be misattributed to hypertension or diabetes mellitus, both of which are more prevalent in racial minorities. 7 …”

“…For instance, while we know that germline expression of the IGLV1-44 gene is associated with cardiac tropism and the IGLV6-57 gene is associated with renal tropism, variances in gene expression with regard to race or ethnicity haven’t been assessed. 7 …”

Modern concepts of primary systemic AL-amyloidosis: the viewpoint of a gastroenterologist

“…Согласно имеющимся статистическим данным, заболеваемость этой формой амилоидоза в западных странах составляет 1 случай на 100000 человеко-лет, при этом системный амилоидоз встречается чаще, чем локальный [7]. Доказано, что расовые и этнические особенности не влияют на распространенность, тяжесть заболевания и эффективность лечения ALамилоидоза [8].…”

Racial disparities in hematopoietic stem cell transplant: a systematic review of the literature

“…The likelihood of obtaining only a partial-HLA match in Hispanics was [OR 1.73 (1.41-2.12)] when compared to white patients in a study by Dehn et al (4), whereas Besse et al (5) showed that Hispanic patients in the 20-25 years old age group had similar odds of finding an HLA-match (40%) when compared to white patients (25-30%). Utilization of HSCT by Hispanic patients was evaluated by three retrospective reviews (6)(7)(8). Costa et al (6) developed a relative utilization rate (RUR) which compared a particular group's utilization of autologous HSCT in multiple myeloma patients as a proportion of the utilization of the entire population.…”