Research Unit @CoeurLab, Centre de Recherche du Centre Hospitalier de l'Université de Montréal (CRCHUM).
Hemato-Oncology division, Département d'Hématologie-Médecine Transfusionnelle du Centre Hospitalier de l'Université de Montréal (CHUM).
Curr Probl Cancer. 2022 Jun;46(3):100833. doi: 10.1016/j.currproblcancer.2021.100833. Epub 2022 Jan 10.
Immunoglobulin light chain (AL) amyloidosis is a complex disease marked by a poor clinical portrait and prognosis generally leading to organ dysfunction and shortened survival. We aimed to review the available evidence on whether AL amyloidosis can lead to malnutrition, thus having a negative impact on quality of life (QoL) and survival.
We searched Pubmed for studies that assessed malnutrition in amyloidosis patients, with no restrictions to the year of publication or language. Retrospective or prospective, observational, and interventional studies that reported data regarding AL amyloidosis and nutritional status were included.
From 62 articles retrieved, 23 were included. Malnutrition was prevalent in up to 65% of patients with AL Amyloidosis. Prevalence of weight loss of 10% or more ranged from 6 to 22% of patients, while a body mass index of < 22 kg/m2 was found in 22 to 42%. Weight loss, lower BMI and other indicators of poor nutritional status were negatively associated with quality of life and survival. Only one RCT focused on nutritional counseling was found and reported positive results on patients QoL and survival.
Despite inconsistencies across assessment criteria, the available data reveal that weight loss and malnutrition are common features in patients with AL amyloidosis. This review reinforces the premise that an impaired nutritional status can be negatively associated with QoL and survival in patients with AL amyloidosis, and therefore should be further investigated.
免疫球蛋白轻链（AL）淀粉样变性是一种复杂的疾病，其临床特征和预后较差，通常导致器官功能障碍和缩短生存时间。我们旨在回顾关于 AL 淀粉样变性是否会导致营养不良的现有证据，从而对生活质量（QoL）和生存产生负面影响。
我们在 Pubmed 上搜索了评估淀粉样变性患者营养不良的研究，对发表年份或语言没有限制。纳入了回顾性或前瞻性、观察性和干预性研究，这些研究报告了与 AL 淀粉样变性和营养状况相关的数据。
从检索到的 62 篇文章中，有 23 篇被纳入。AL 淀粉样变性患者中营养不良的患病率高达 65%。体重减轻 10%或更多的患病率在 6%至 22%之间，而 BMI<22kg/m2 的患病率在 22%至 42%之间。体重减轻、较低的 BMI 和其他营养不良指标与生活质量和生存呈负相关。仅发现了一项关注营养咨询的 RCT，该研究报告了对患者 QoL 和生存的积极结果。
尽管评估标准存在差异，但现有数据表明，体重减轻和营养不良是 AL 淀粉样变性患者的常见特征。这篇综述强调了一个前提，即营养状况受损可能与 AL 淀粉样变性患者的 QoL 和生存呈负相关，因此应进一步研究。
