Division of Hematology/Oncology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, 53226, USA.
Division of Biostatistics, Institute of Health and Equity, Medical College of Wisconsin, Milwaukee, WI, 53226, USA.
Blood Cancer J. 2024 May 26;14(1):61. doi: 10.1038/s41408-024-01040-8.
It is well-established that most patients with systemic light chain (AL) amyloidosis have multi-organ involvement and are often diagnosed after a lag period of increasing symptoms. We leverage electronic health record (EHR) data from the TriNetX research network to describe the incidence, timing, and co-occurrence of precursor conditions of interests in a cohort of AL amyloidosis patients identified between October 2015-December 2020. Nineteen precursor diagnoses of interest representing features of AL amyloidosis were identified using ICD codes up to 36 months prior to AL amyloidosis diagnosis. Among 1,401 patients with at least 36 months of EHR data prior to AL amyloidosis diagnosis, 46% were females, 16% were non-Hispanic Black, and 6% were Hispanic. The median age was 71 (range, 21-91) years. The median number of precursor diagnoses was 5 with dyspnea and fatigue being the most prevalent. The time from the first occurrence of a precursor to AL diagnosis ranged from 3.2 to 21.4 months. Analyses of pairwise co-occurrence of specific diagnoses indicated a high association (Cole's coefficient >0.6) among the examined precursor diagnoses. These findings provide novel information about the timing and co-occurrence of key precursor conditions and could be used to develop algorithms for early identification of AL amyloidosis.
众所周知,大多数系统性轻链(AL)淀粉样变性患者存在多器官受累,并且常在症状逐渐加重一段时间后才被诊断出来。我们利用 TriNetX 研究网络中的电子健康记录(EHR)数据,描述了 2015 年 10 月至 2020 年 12 月期间确诊的 AL 淀粉样变性患者队列中,感兴趣的前驱疾病的发病情况、时间和同时发生情况。使用 ICD 编码,在 AL 淀粉样变性诊断前最多提前 36 个月确定了 19 个感兴趣的前驱诊断。在至少有 36 个月的 AL 淀粉样变性诊断前 EHR 数据的 1401 名患者中,46%为女性,16%为非西班牙裔黑人,6%为西班牙裔。中位年龄为 71 岁(范围为 21-91 岁)。中位数的前驱诊断数为 5 个,呼吸困难和疲劳最为常见。从前驱疾病首次发生到 AL 诊断的时间范围为 3.2 至 21.4 个月。对特定诊断的配对共现分析表明,检查的前驱诊断之间存在高度关联(Cole 系数>0.6)。这些发现提供了有关关键前驱疾病的发生时间和同时发生情况的新信息,可用于开发早期识别 AL 淀粉样变性的算法。