| Background and objective:Systemic light chain amyloidosis(AL)is a kind of monoclonal plasma cell disease with multisystem involvement.The disease is lack of typical clinical manifestations and low morbidity,so it is easy to be misdiagnosed in clinical work and delay treatment.Early diagnosis and treatment are very important for the prognosis of AL.This study summarizes the clinical features,treatment and prognosis of newly treated AL in our hospital,so as to deepen the understanding of the disease and provide more experience for the diagnosis and treatment of the disease.Methods:The clinical characteristics,treatment and prognosis of 49 patients with primary AL admitted to the Department of Hematology,First Hospital of Jilin University from January 1,2012 to December 31,2019 were retrospectively analyzed.The measurement data of normal distribution were expressed as mean ± standard deviation,the measurement data of non-normal distribution were expressed as median(quartile spacing),and the classified variables were expressed as frequency(constituent ratio).T-test was used to compare the measurement data of normal distribution,rank sum test was used to compare the measurement data of non-normal distribution,and chi-square test was used to compare the classified variables between groups.Univariate survival analysis was carried out by Kaplan-Meier method,and survival curve was drawn.Log-rank test was used to compare the survival curve.The variables with P <0.1 in univariate analysis were included in multivariate COX regression model analysis.The above data analysis was completed by SPSS 24.0statistical software,and the drawing was completed by Graph Pad Prism 5.P < 0.05 was considered a statistically significant difference.Results:(1)Among the 49 patients with AL,there were 33 males(67.3%)and 16 females(32.7%).The average age was 58.33 ± 8.94 years old,and the patients over 60 years old accounted for 49.0%.Among the 49 patients with AL,there were 41 cases of type λ(83.7%)and 8 cases of type κ(16.3%).Kidney was involved in 39 cases(79.6%),heart in 34 cases(69.4%),skin in 12 cases(24.5%),liver in 5 cases(10.2%),nervous system in 3 cases(6.1%),tongue in 2 cases(4.1%),gastrointestinal tract in 1 case(2.0%).(2)The positive rates of M protein detected by serum immunofixation electrophoresis,urine immunofixation electrophoresis,serum protein electrophoresis and serum free light chain ratio abnormal method were 38.3%(18/47),46.8%(22/47),30.2%(13/43)and 83.7%(41/49),respectively.The combination of the above four methods can increase the positive rate of M protein to 100.0%.(3)Among the 49 patients,the clinical indexes of type λ and type κ were compared and analyzed,and the patients with type λ had higher troponin I than those with type κ(P=0.022).There was no statistical difference in age,N-terminal pronatriuretic peptide(NT-proBNP)and interventricular septal thickness between the two groups.(4)The treatment regimens of 49 patients were divided into 4 groups: transplantation group,chemotherapy ≤2 courses group,chemotherapy >2 courses group,and palliative care group.There was a statistical difference in overall survival(OS)time between the different treatment regimen groups(P=0.001).Patients treated with transplant or chemotherapy were assessed for efficacy and there was a statistical difference in OS between the hematological efficacy assessment ≥very good partial remission(VGPR)and <VGPR groups(P=0.02).(5)In the univariate survival analysis,age ≥ 60 years,NT-proBNP ≥ 1800 pg/ml,and NYHA classification ≥ 3 were risk factors affecting OS in AL patients.Factors with P < 0.1in the univariate were included in the COX multifactor analysis,and age ≥ 60 years and NT-proBNP ≥ 1800 pg/ml were independent risk factors affecting OS in patients with AL.Conclusions:(1)AL was more common in the middle-aged and elderly,male was more than female,and λ type was more common.The clinical manifestations were lack of typicality,mainly involving kidney,heart,,which were easy to be misdiagnosed in the early stage.(2)For patients with suspicious clinical manifestations of AL,serum immunofixation electrophoresis,urine immunofixation electrophoresis,serum protein electrophoresis,and serum free light chain should be improved promptly for screening.(3)Once diagnosed with AL,patients should be considered for autologous stem cell transplantation(ASCT)or systemic chemotherapy with the aim of achieving a hematologic efficacy assessment of VGPR or above.(4)Multivariate survival analysis showed that age ≥ 60 years and NT-proBNP ≥ 1800pg/ml were independent risk factors for OS in AL patients. |
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