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目的:探讨伴超高水平血清游离轻链（FLC）的轻链型（AL型）淀粉样变患者的临床特征及预后。方法:回顾性分析2009年1月至2020年1月北京协和医院确诊的595例AL型淀粉样变患者的临床资料，按FLC水平将其分为两组：超高FLC组[FLC差值（dFLC）>500 mg/L，124例]与非超高FLC组（dFLC≤500 mg/L，471例），比较两组患者的临床特征和预后。结果:超高FLC组患者与非超高FLC组相比，心脏受累比例更高（82.3%对70.1%，n P=0.007），2004梅奥分期Ⅲ期比例更高（41.8%对33.8%，n P=0.029），肾脏受累比例更低（59.7%对71.8%，n P=0.009）。超高FLC组患者与非超高FLC组相比，血液学缓解率降低（72.4%对82.3%，n P=0.048），心脏缓解率降低（37.3%对54.7%，n P=0.016），中位总生存期缩短（13.0个月对未达到，n P<0.001），3个月内早期死亡率升高（28.2%对11.3%，n P500 mg/L是影响AL型淀粉样变患者预后的独立危险因素（n HR=2.279，95%n CI 1.685~3.083，n P500 mg/L; n n=124] and those without ultra-high FLC levels (dFLC≤500 mg/L; n n=471) .n Results:Patients with ultra-high FLC presented with more frequent cardiac involvement (82.3% n vs 70.1%, n P=0.007) , and a higher percentage of patients with 2004 Mayo Ⅲ stage (41.8% n vs 33.8%, n P=0.029) , but less frequent renal involvement than patients without an ultra-high FLC (59.7% n vs 71.8%, n P=0.009) . Patients with an ultra-high FLC achieved a lower proportion of hematologic (72.4% n vs 82.3%, n P=0.048) and cardiac response (37.3% n vs 54.7%, n P=0.016) and had shorter overall survival (13.0 months n vs not reached, n P<0.001) and a higher early death rate within 3 months (28.2%n vs 11.3%, n P<0.001) than those without an ultra-high FLC. Ultra-high FLC independently predicted worse prognosis in patients with AL amyloidosis (n HR=2.279, 95%n CI 1.685-3.083, n P<0.001) .n Conclusions:Patients with an initially ultra-high FLC represented a subgroup with more common cardiac involvement, more advanced cardiac stages, and extremely poor prognosis.