The clinical features and outcomes of systemic AL amyloidosis: a cohort of 231 Chinese patients

摘要: Background. Few data are available on the clinical features and outcomes of Chinese patients with systemic immunoglobulin light-chain (AL) amyloidosis. The aim this study is to reveal picture risk factors disease progression in a large cohort AL Methods. Patients Jinling Hospital amyloidosis registry from 2003 2011 were studied. laboratory information collected first presentation death or until last follow-up. patients’ survival renal analyzed, relationships between parameters also assessed. Results. A total 231 enrolled study, all studied had involvement. One hundred fifty-three (66.2%) male, median age at diagnosis was 56 years. 198 (85.7%) cases λ-type. forty-seven (63.6%) presented as nephrotic syndrome (NS), 25% insufficiency diagnosis. Liver involvement NS appeared be more common κ-type amyloidosis, impairment severe time 36.3 months, 1-, 2-, 3- 5-year cumulative rates 67, 53, 48 35%, respectively. Multivariate COX analysis showed that age, hepatic heart can significantly influence these patients. remained dialysis free 50 months. percentage 1, 2, 3 5 years 78, 69, 62 37%, serum creatinine hypotension important failure. Conclusion. λ-Type most dominant type poor. included involvement, function. high level associated poor outcome.