| Objective: AL amyloidosis is a protein disfolding disease,with low morbidity and high mortality.The large cohort epidemiology study of AL amyloidosis in China is rare.The non-invasive or minimal-invasive methods of diagnosis and the biomarkers of prognosis and treatment effect are absence.Some study indicated that vitronectin colocalizes with the tissue amyloidosis deposition,while the underlying mechanism of vitronectin is unknown,the circulation level of vitronectin in AL amloidosis hasn't been studied.The aim of our study is to reveal the clinical-pathological features and prognostic factors in a large cohort of AL amyloidosis in the Northwest of China,and investigate the circulation level of vitronectin in the diagnosis and prognosis of AL amloidosis.Methods: The patients diagnosed as AL amyloidosis during Jan.2003 to Sep.2017 in Xijing Hospital were studied retrospectively.The detailed clinical,pathological and laboratory data,the treatment regimens and the follow-up data were collected and analyzed.Serum vitronectin level of AL amyloidosis and control groups is detected by double-antibody sandwich ELISA.Results: 164 patients of AL amyloidosis were enrolled,and 130 were diagnosed by renal biopsy.The mean age was 57.5±9.8 years old and 62.8%(103/164)were male.The most common symptom was edema,92.7% of the patients had the kidney involved and 50% patients had the heart involved,73.2%(120)patients had more than one organ involved.24.8% patients were classified as Mayo clinic Stage?,31.0% patients were Stage ? and 37.9% were Stage ?,6.2% were stage ?.The median survival time of patients untreated is 13 months,and the 1 year,2 year,3 year,5 year survival rate are 56.5%?45.9%?30.6%?23.6% respectively.The 1 year and 2 year survival rate of patients treated are 94.6%?92.5% respectively.The heart involvement(OR 2.586,95%CI: 1.355-4.936),and hypotension(OR 1.870,95%CI: 1.039-3.35)were independent risk factors of prognosis and the chemotherapy(OR 0.344,95% CI: 0.133-0.893)was the protective factor of prognosis.30 AL amyloidosis patients were enrolled in study group.The serum vitronenctin concentration of untreated AL amyloidosis group is significantly higher than healthy control group and chronic glomerulonephritis group(74103.9ng/ml vs.53946.3ng/ml,p=0.008;74103.9ng/ml vs 42240.3ng/ml,p<0.001 respecitively).Serum vitronentin concentration of AL amyloidosis who had achieved hematologic response(VGPR or better)significantly decreases(81341.1ng/ml vs 36159.9ng/ml,p=0.033).ROC analysis indicated that,the threshold value of serum VTN concentration 66797.8ng/ml will predicate AL amyloidosis with 92.7% specificity and 66.7% sensitivity.Conclusion: Aged people are more frequently involved in AL Amyloidosis,and the edema and kidney involvement are most common.The median survival time of patients untreated is 13 months.The survival rate of patients after chemotherapy increase significantly.The heart involvement and hypotension were independent risk factors of prognosis.The serum vitronenctin concentration of AL amyloidosis group is significantly higher than control group.Serum vitronentin concentration decreases along with remission of AL amyloidosis.The threshold value of serum VTN concentration 66797.8ng/ml will predicate AL amyloidosis with high specificity.The vitronenctin maybe a new biomarker for differential diagnosis and evaluating therapeutic effects of AL amyloidosis,and the underlying mechanism should be investgated further. |
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