| Objective To investigate the clinical characteristics,reasonable treatment strategies and prognostic factors of adult anaplastic large cell lymphoma(ALCL).Method The data of 33 adult patients with ALCL diagnosed in the first affiliated Hospital of Guangxi Medical University from January 1,2010 to October 1,2018 were analyzed retrospectively.The clinical characteristics,therapeutic effect and prognosis were analyzed retrospectively.Result1.Among the 33 patients,there were 21 males(63.6%)and 12 females(36.4%).The ratio of male to female was 1.75:1.The median age of onset was 38 years(16-56 years)in ALK + ALCL patients(18 cases VS 15 cases).The median age of onset in ALK+ ALCL group was 28 years old(16-51 years old),and the median age of onset in ALK-ALCL group was 45 years old(18-56 years old).In this study,most of the patients with ALK+ ALCL were less than 40 years old(14/18,77.8%),and most of the patients with ALK-ALCL were more than 40 years old(10/15,66.7%).2.B symptom(23/33)was the most common in 33 patients,of which fever was the most common(54.55%),and hemophagocytic syndrome was found in 4 patients with fever.The second were enlarged lymph nodes,abdominal pain,cough,nausea and vomiting,intestinal obstruction,dyspnea and other unusual clinical manifestations,including skin pruritus,acid belching and nasal congestion.66.7% of the patients were diagnosed as advanced stage(?/? stage),and 54.5% of the patients were diagnosed as high school risk group / high risk group.3.The 1-year OS and 3-year OS of 24 patients with standardized treatment were 79% and 71% respectively,and the 1-year PFS and 3-year PFS were 70% and 65%,respectively.The 5-year OS of ALK+ ALCL patients was 83%,while that of ALK-ALCL patients was 61%.4.The initial treatment regimens of 24 adult ALCL patients were CHOP(n=7),CHOP+ etoposide(n=15),Hyper CVAD(n=2).The 1-year OS in CHOP group and CHOP+ etoposide group were 57% and 86%,and the 3-year OS were 57% and 72%,respectively.OS in CHOP+ etoposide group was better than that in CHOP alone group,but the effect of treatment on survival was not statistically significant(P=0.2437).In the mid-term evaluation,ORR was 57.1%(4/7)in CHOP chemotherapy group,93.3%(14/15)in CHOP+ etoposide chemotherapy group,92.3% in 13 ALK+ ALCL patients and 63.6% in 11 ALK-ALCL patients.Two patients with ALK-ALCL were treated with chemotherapy combined with autologous hematopoietic stem cell transplantation and are still alive.5.Univariate analysis showed that the increase of serum ? 2-MG,the increase of tumor marker CA125 and age ? 40 years were the adverse prognostic factors for the survival of ALCL patients(P = 0.015,0.049 and 0.013,respectively).Multivariate analysis showed that the increase of serum ? 2-MG was an independent prognostic factor for patients with OS(P < 0.037,HR 2.648,95% CI: 1.063 6.597).Conclusion1.Adult ALCL is more common in male patients,mostly in late stage(?/? stage),ALK+ ALCL patients are more common,ALK+ ALCL is more common in young patients < 40 years old,ALK-ALCL is more common in middle-aged and elderly patients,clinical manifestations of B symptoms are the most common,lack of specificity.2.The prognosis of ALK+ ALCL was better than that of ALK-ALCL.The 5-year OS was 83% and 61%,respectively.Etoposide may be beneficial in patients with ALCL under 60 years old,and hematopoietic stem cell transplantation may be an effective method for poor prognosis or refractory/recurrent ALCL patients.3.The increase of serum ? 2-MG level is the independent prognostic factor affecting the prognosis of patients. |
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