| ObjectiveThis paper aims to investigate the clinical characteristics of anaplastic large cell lymphoma(ALCL)and prognostic factors affecting progression-free survival(PFS)and overall survival(OS)of patients.MethodA retrospective analysis was conducted on 67 ALCL cases in Fujian Medical University Union Hospital from December 2009 to December 2018.The demographic characteristics,clinical characteristics,laboratory indices and immunohistochemical characteristics were recorded,and their clinical and laboratory indices and immunohistochemical results were analyzed by prognosis-related single-factor analysis.ResultOf the 67 patients,there were 40 males(59.7%)and 27 females(40.3%),with the ratio of male: female was 1.48:1.The median age was 20(0.8-76)years old,the median age in positive anaplastic lymphoma kinase(ALK)patients was 17(0.8-65)years old,and the median age in negative ALK patients was 51(23-76)years old.In adult’s(≥14 years old)Ann Arbor staging,there were 2 cases(4.3%)in Stage I,6 cases(13.0%)in Stage II,20 cases(43.5%)in Stage III and 18 cases(39.1%)in Stage IV.In children’s(<14 years old)St.Jude staging,there were 5 cases(23.8%)in Stage II,9 cases(42.9%)in Stage III and 7 cases(33.3%)in Stage IV.There were 43 cases(69.4%)with symptom B,and 26 cases(43.3%)with an LDH level higher than normal;62 cases(92.5%)in primary systemic type and 5 cases(7.4%)in primary cutaneous type;17 cases(50.0%)with ≥2 extracapsular extensions in initial treatment,12 cases(20.0%)with bone marrow involvement;51 cases(77.3%)with positive ALK and 15 cases(22.7%)with negative ALK.The positive rates of EMA(P=0.001),TIA(P=0.046)and granzyme B(P=0.003)in the ALK positive group were significantly higher than those in the ALK negative group.In most of ALCL cases,CD10,CD15,CD20,PAX-5,Td T and EBER were not expressed,and Ki-67 was highly expressed in tumor cells.The overall response rate(ORR)was 74.5%,and the ORR of ALK+ALCL and ALK-ALCL were 82.5% and 50.0%.In adult group,the ORR of CHOP±E chemotherapy group was 76.0%.The 5-year survival rate of ALCL and the 5-year progression-free survival rate of ALCL were 77.4% and 73.6%,the 5-year average PFS was 46.30(40.25-52.34)months and the 5-year average OS was49.01(43.42-54.60)months.The death of patients was mainly in the first 14 months after diagnosis.The OS of systemic anaplastic large cell lymphoma(s ALCL)with positive ALK was significantly longer than that of s ALCL with negative ALK(P=0.020),and the PFS(P=0.090)had no statistically significant difference.In ALK positive s ALCL,the 5-year average PFS was 48.89(42.36-55.41)months,the5-year average OS was 52.52(46.93-58.11)months,the 5-year survival rate was86.9%,and the 5-year progression-free survival rate were 79.6%.In ALK negative s ALCL,the 5-year average PFS was 34.20(17.96-50.45)months,the 5-year average OS was 34.60(18.61-50.59)months,the 5-year survival rate and the 5-year progression-free survival rate were both 50.0%.In s ALCL,the OS of ALK positive group was significantly longer than that of ALK negative group.Univariate analysis showed that age≥60 years old(P=0.001)and the loss of the granzyme B(P=0.046)were the adverse prognostic factors for the survival of ALCL.Age(P=0.005),accompanied symptom B(P=0.031),bone marrow involvement(P=0.027)and the loss of granzyme B(P=0.046)were the adverse prognostic factors for the PFS of ALCL.Conclusion1.Among ALCL patients,the incidence rate of males was higher than that of females.ALK+ALCL was more common in people aged 30 years and below,and ALK-ALCL was more common in people aged above 20 years,with a relatively high proportion of patients in Stage III-IV accompanied symptom B and in positive ALK classification.The positive rates of EMA,TIA and granzyme B in the ALK positive ALCL group were significantly higher than those in the ALK negative ALCL group.In most of ALCL cases,EBER were not expressed,and Ki-67 was highly expressed in tumor cells.2.The survival of ALK positive s ALCL was significantly longer than that of ALK negative s ALCL.Univariate analysis showed that age≥60 years old and the loss of granzyme B were the adverse prognostic factors for the survival of ALCL,and age≥60 years old,accompanied symptom B,bone marrow involvement and the loss of granzyme B were the adverse prognostic factors for the PFS of ALCL. |
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