National Amyloidosis Centre, Centre for Amyloidosis & Acute Phase Proteins, University College London, UK.
Haematologica. 2013 Jan;98(1):136-40. doi: 10.3324/haematol.2012.070359. Epub 2012 Sep 14.
Weight loss is common in systemic immunoglobulin light chain amyloidosis but there are limited data on the impact of nutritional status on outcome. Using the Patient-Generated Subjective Global Assessment (PG-SGA) score, we prospectively examined nutritional status in 110 consecutive newly-diagnosed, treatment-naïve patients with immunoglobulin light chain amyloidosis attending the UK National Amyloidosis Centre. At study entry, 72 of 110 (66%) patients had a PG-SGA score of 4 or over, indicating malnutrition requiring specialist nutritional intervention. Number of amyloidotic organs, elevated alkaline phosphatase, presence of autonomic neuropathy and advanced Mayo disease stage were independently associated with poor nutritional status (P<0.05). Quality of life was substantially poorer among those with higher PG-SGA scores (P<0.001). Furthermore, PG-SGA score was a powerful independent predictor of patient survival (P=0.02). Malnutrition is prevalent and is associated with poor quality of life and reduced survival among patients with systemic immunoglobulin light chain amyloidosis. The PG-SGA score would be an appropriate tool to evaluate whether nutritional intervention could improve patient outcomes.
体重减轻在系统性免疫球蛋白轻链淀粉样变性中很常见,但关于营养状况对预后的影响的数据有限。我们使用患者生成的主观整体评估(PG-SGA)评分,前瞻性地检查了 110 例新诊断、未经治疗的免疫球蛋白轻链淀粉样变性患者的营养状况,这些患者均在英国国家淀粉样变性中心就诊。在研究开始时,110 例患者中有 72 例(66%)PG-SGA 评分为 4 分或以上,表明存在营养不良,需要专业的营养干预。淀粉样变性器官的数量、碱性磷酸酶升高、自主神经病的存在和 Mayo 疾病晚期是与不良营养状况相关的独立因素(P<0.05)。PG-SGA 评分较高的患者生活质量明显较差(P<0.001)。此外,PG-SGA 评分是患者生存的有力独立预测因子(P=0.02)。营养不良在系统性免疫球蛋白轻链淀粉样变性患者中很常见,与生活质量差和生存率降低有关。PG-SGA 评分将是评估营养干预是否可以改善患者预后的合适工具。